Strain Name:

FVB.129P2-Pde6b+ Tyrc-ch Fmr1tm1Cgr/J

Stock Number:

004624

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Availability:

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These Fmr1 KO mice may be useful for studying behavioral and synaptic abnormalities associated with Fragile X Syndrome.

Description

Strain Information

Former Names FVB.129P2-Fmr1tm1Cgr/J    (Changed: 27-JAN-12 )
Type Congenic; Mutant Strain; Spontaneous Mutation; Targeted Mutation;
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Mating SystemHomozygote x Hemizygote         (Female x Male)   01-MAR-06
Specieslaboratory mouse
GenerationN11+1F21 (27-NOV-11)
Generation Definitions
 
Donating Investigator William T. Greenough,   Beckman Institute

Important Note
This Fmr1 knockout is also available on a C57BL/6 genetic background: B6.129P2-Fmr1tm1Cgr/J (Stock No. 003025).

Description
   Hemizygous male and homozygous female mice for this knockout show many phenotypic characteristics of the Fragile X Syndrome in humans that lack the fragile X mental retardation protein (FMRP) as a result of a mutation in the FMR1 gene. FMRP is an RNA binding protein whose function is shown to be involved in translational regulation of specific dendritic mRNAs. Certain regions of the brain in these mice are characterized by the presence of long, thin dendritic spines on excitatory neurons.
   Behavioral traits include deficits in classical delay eye-blink conditioning, autistic-like core symptoms of altered social interaction and occurrence of repetitive behaviors with additional hyperactivity, reduced anxiety, and increased errors in a learning assay. Whole-cell patch-clamp recordings in the anterior cingulate cortex show that long-term potentiation is completely abolished. A similar decrease in long-term potentiation is found in the lateral amygdala, another structure along with the anterior cingulate cortex implicated in fear memory. Failure of the startle response to develop after 4th postnatal week is seen.
   Cellular defects include abnormalities in neurogenesis that are seen in the embryonic FMRP-deficient brain; neural progenitors accumulate abnormally in the subventricular zone of the embryonic neocortex.
   Abnormalities of dendritic spines in multiple regions of the brain of Fmr1-knockout mice are seen; absence of FMRP induces an over-activation of RAC1, a protein of the Rho GTPase subfamily that plays a critical role in dendritic morphology and synaptic function. Inhibitory synaptic abnormalities in the amygdala as a result of defective GABAergic neurotransmission have also been reported.
   The absence of FMRP also causes defects of protein synthesis-dependent plasticity seen as an impairment of long-term potentiation in the cortex and hippocampus, as well as an augmentation of long-term depression in the hippocampus and cerebellum. Presynaptic abnormalities at excitatory hippocampal synapses in the knock-out mice also lead to defects in short-term plasticity and information processing.
   Studies of Fmr1-KOs have revealed that over-activation of class I metabotropic glutamate receptor signaling is a primary defect in the cerebral cortex and hippocampus affecting synaptic plasticity. Enhanced tyrosine kinase B (TrkB) expression and brain derived neurotrophic factor (BDNF)-induced intracellular calcium responses in cortical neural progenitor cells lacking FMRP as well as changes in the expression of TrkB are seen in embryonic Fmr1-KO mice. Mammalian target of rapamycin (mTOR) phosphorylation and activity are elevated in the hippocampus of juvenile Fmr1-KO mice. Brains from Fmr1-KO mice display higher levels of reactive oxygen species, nicotinamide adenine dinucleotide phosphate (NADPH)-oxidase activation, lipid peroxidation and protein oxidation than brains from wild-type mice.
   Mice that are hemizygous or homozygous for the targeted mutation are viable and fertile. These mice possess the wildtype Pde6b allele and do not suffer from blindness due to retinal degeneration. This mutant mouse strain has proven useful in studies related to Fragile X Syndrome.

Development
A targeting vector containing neomycin resistance was used to disrupt exon 5 of the targeted gene. The construct was electroporated into 129P2/OlaHsd derived E14 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6 blastocysts. Mice were backcrossed for 11 generations onto the FVB background, and are homozygous for the 129P2/OlaHsd wildtype Pde6b allele.

Control Information

  Control
   See control note: The control for this strain is STOCK# 4828, FVB.129P2-Pde6b+ Tyrc-ch/AntJ.
   004828 FVB.129P2-Pde6b+ Tyrc-ch/AntJ
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Fmr1tm1Cgr allele
003025   B6.129P2-Fmr1tm1Cgr/J
003024   FVB.129P2(B6)-Fmr1tm1Cgr/J
002700   FVB;129P-Fmr1tm1Cgr/J
View Strains carrying   Fmr1tm1Cgr     (3 strains)

View Strains carrying   Pde6b+     (10 strains)

Strains carrying   Tyrc-ch allele
000091   129T1/Sv-Oca2+ Tyrc-ch Dnd1Ter/J
000578   B6 x STOCK Tyrc-ch Bmp5se +/+ Myo6sv/J
000619   FS/EiJ
004828   FVB.129P2-Pde6b+ Tyrc-ch/AntJ
000271   SH1/LeJ
000306   STOCK Dll3pu + Tyrc-ch/+ Oca2p Tyrc-ch/J
View Strains carrying   Tyrc-ch     (6 strains)

Strains carrying other alleles of Fmr1
010504   B6.129-Fmr1tm1Rbd/J
008909   FVB.129-Fmr1tm1Rbd/J
View Strains carrying other alleles of Fmr1     (2 strains)

Strains carrying other alleles of Pde6b
004202   B6.C3 Pde6brd1 Hps4le/+ +-Lmx1adr-8J/J
000002   B6.C3-Pde6brd1 Hps4le/J
004297   B6.CXB1-Pde6brd10/J
001022   B6C3FeF1/J a/a
000652   BDP/J
000653   BUB/BnJ
002439   C3.129P2(B6)-B2mtm1Unc/J
005494   C3.129S1(B6)-Grm1rcw/J
000509   C3.Cg-Lystbg-2J/J
000480   C3.MRL-Faslpr/J
001957   C3A Pde6brd1.O20/A-Prph2Rd2/J
004326   C3Bir.129P2(B6)-Il10tm1Cgn/Lt
003968   C3Bir.129P2(B6)-Il10tm1Cgn/LtJ
006435   C3Fe.SW-Soaa/MonJ
001904   C3H-Atcayji-hes/J
000659   C3H/HeJ
000511   C3H/HeJ-Ap3d1mh-2J/J
000784   C3H/HeJ-Faslgld/J
002433   C3H/HeJ-Sptbn4qv-lnd2J/J
005972   C3H/HeJBirLtJ
001824   C3H/HeJSxJ
000635   C3H/HeOuJ
000474   C3H/HeSn
001431   C3H/HeSn-ocd/J
000661   C3H/HeSnJ
002333   C3H/HeSnJ-gri/J
001576   C3He-Atp7btx-J/J
000658   C3HeB/FeJ
002588   C3HeB/FeJ-Eya1bor/J
001533   C3HeB/FeJ-Mc1rE-so Gli3Xt-J/J
001908   C3HfB/BiJ
001502   C3Sn.B6-Epha4rb/EiGrsrJ
002235   C3Sn.C3-Ctnna2cdf/J
001547   C3Sn.Cg-Cm/J
001906   C3fBAnl.Cg-Catb/AnlJ
004766   C57BL/6J-Pde6brd1-2J/J
000656   CBA/J
000813   CBA/J-Atp7aMo-pew/J
000660   DA/HuSnJ
000023   FL/1ReJ
000025   FL/4ReJ
003024   FVB.129P2(B6)-Fmr1tm1Cgr/J
002539   FVB.129P2-Abcb4tm1Bor/J
002935   FVB.129S2(B6)-Ccnd1tm1Wbg/J
002953   FVB.Cg-Tg(MMTVTGFA)254Rjc/J
003170   FVB.Cg-Tg(Myh6-tTA)6Smbf/J
003078   FVB.Cg-Tg(WapIgf1)39Dlr/J
003487   FVB.Cg-Tg(XGFAP-lacZ)3Mes/J
003257   FVB/N-Tg(GFAPGFP)14Mes/J
002856   FVB/N-Tg(TIE2-lacZ)182Sato/J
002384   FVB/N-Tg(UcpDta)1Kz/J
001800   FVB/NJ
001491   FVB/NMob
000804   HPG/BmJ
000734   MOLD/RkJ
000550   MOLF/EiJ
002423   NON/ShiLtJ
000679   P/J
000680   PL/J
000268   RSV/LeJ
000269   SB/LeJ
010968   SB;C3Sn-Lrp4mdig-2J/GrsrJ
005651   SJL.AK-Thy1a/TseJ
000686   SJL/J
000688   ST/bJ
004808   STOCK Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J
002648   STOCK a/a Cln6nclf/J
000279   STOCK gr +/+ Ap3d1mh/J
005965   STOCK Tg(Pomc1-cre)16Lowl/J
004770   SW.B6-Soab/J
002023   SWR.M-Emv21 Emv22/J
000689   SWR/J
000939   SWR/J-Clcn1adr-mto/J
000692   WB/ReJ KitW/J
100410   WBB6F1/J-KitW/KitW-v/J
000693   WC/ReJ KitlSl/J
View Strains carrying other alleles of Pde6b     (76 strains)

Strains carrying other alleles of Tyr
000090   129S1/Sv-Oca2+ Tyr+ KitlSl-J/J
005445   A.B6 Tyr+-Cybanmf333/J
005012   A.B6 Tyr+-Myo5ad-l31J/J
002565   A.B6-Tyr+/J
001017   AKXD10/TyJ
000765   AKXD13/TyJ
000954   AKXD15/TyJ
000958   AKXD16/TyJ
001093   AKXD18/TyJ
001062   AKXD21/TyJ
000947   AKXD22/TyJ
000969   AKXD24/TyJ
000777   AKXD6/TyJ
000763   AKXD9/TyJ
000409   B10.129P-H1b Hbbd Tyrc Ea7a/(5M)oSnJ
000418   B10.129P-H1b Tyrc Hbbd/(5M)nSnJ
000432   B10.C-H1b Hbbd Tyrc/(41N)SnJ
000580   B10.D2/nSn-Tyrc-4J/J
000822   B6 x 129S1/SvEi Oca2+ Tyr+-Vsx2or-J/J
017614   B6(Cg)-Tyrc-2J Tg(UBC-mCherry)1Phbs/J
000058   B6(Cg)-Tyrc-2J/J
008647   B6.129P2(Cg)-Trpa1tm1.1Kykw Tyrc-2J/J
000383   B6.C-Tyrc H1b Hbbd/ByJ
013590   B6.Cg-Braftm1Mmcm Ptentm1Hwu Tg(Tyr-cre/ERT2)13Bos/BosJ
003819   B6.Cg-Per2tm1Brd Tyrc-Brd/J
023429   B6.Cg-Tyrc-2J Cdkn1atm1Hpw/J
007484   B6.Cg-Tyrc-2J Tg(Tyr)3412ARpw Tg(Sry-EGFP)92Ei/EiJ
000035   B6.Cg-Tyrc-J/J
000104   B6.Cg-Tyrc-h/J
005349   B6.Cg-awag Tyrc-2J/GrsrJ
012328   B6.Cg-Tg(Tyr-cre/ERT2)13Bos/J
000054   B6.D2-Tyrc-p/J
023428   B6;129X1-Tyrc-2J Cdkn1atm2Hpw/J
000899   C.B6-Tyr+ Hbbs/J
000339   C3H/HeJ-Tyrc-9J/J
001294   C3H/HeJ-Tyrc-a/J
001002   C57BL/10SnJ-Tyrc-11J/J
021999   C57BL/6NJ-Tyrem3J/GrsrJ
012257   CB6-Tg(Tyr-TAg)BJjw/Mmjax
001006   CBA/J-Tyrc-10J/J
000657   CE/J
007483   FVB.Cg-Tg(Tyr)3412ARpw Tg(Sry-EGFP)92Ei/EiJ
017387   FVB/N-Abce1Tg(Tyr)2295G-2b3Ove/Mmjax
017378   FVB/N-AclyTgTn(sb-cHS4,Tyr)2517BOve/Mmjax
017416   FVB/N-Atad2bTg(Tyr)2295B-4Ove/Mmjax
017369   FVB/N-Casc5Tg(Tyr)2397HOve/Mmjax
017411   FVB/N-CbfbTgTn(sb-cHS4,Tyr)2512E-2Ove/Mmjax
017389   FVB/N-Ccdc174Tg(Tyr)2401AOve/Mmjax
017398   FVB/N-Cct3Tg(Tyr)2423AOve/Mmjax
017363   FVB/N-Chchd3TgTn(sb-cHS4,Tyr)2503C-1bOve/Mmjax
017437   FVB/N-Ckap5TgTn(sb-cHS4,Tyr)2320F-1Ove/J
017415   FVB/N-Cks2TgTn(sb-cHS4,Tyr)2525F-2Ove/Mmjax
017418   FVB/N-Cnpy4Tg(Tyr)2356C-2a1Ove/Mmjax
016870   FVB/NJ-Ap2b1Tg(Tyr)427Ove/EtevJ
000494   J.Cg-Oca2+ Tyr+ Lystbg/J
002281   NFS.C58-Tyr+/J
004304   NOD.CBALs-Tyr+/LtJ
001759   STOCK A Tyrc Sha/J
018129   STOCK Fah1R Tyrc/RJ
000006   STOCK Hk Tyrc/J
014173   STOCK Omptm1.1(COP4*/EYFP)Tboz/J
000206   STOCK a/a Tyrc-h/J
View Strains carrying other alleles of Tyr     (62 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Fragile X Mental Retardation Syndrome
- Model with phenotypic similarity to human disease where etiologies are distinct. Human genes are associated with this disease. Orthologs of these genes do not appear in the mouse genotype(s).
Autism
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Albinism, Ocular, with Sensorineural Deafness   (TYR)
Albinism, Oculocutaneous, Type IA; OCA1A   (TYR)
Albinism, Oculocutaneous, Type IB; OCA1B   (TYR)
Fragile X Tremor/Ataxia Syndrome; FXTAS   (FMR1)
Premature Ovarian Failure 1; POF1   (FMR1)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Fmr1tm1Cgr/Y

        FVB.129P2(B6)-Pde6b+ Fmr1tm1Cgr
  • behavior/neurological phenotype
  • abnormal associative learning   (MGI Ref ID J:100197)
    • impaired cued conditioning behavior
      • mice exhibit reduced freezing compared to controls following CS-US pairings in trace fear conditioning paradigm   (MGI Ref ID J:100197)
      • mice exhibit reduced average freezing within the intertrial intervals   (MGI Ref ID J:100197)
      • there are no significant differences in locomotor activity, nociceptive responses and anxiety-like behaviors between mutants and controls   (MGI Ref ID J:100197)
  • nervous system phenotype
  • reduced long term potentiation
    • synaptic potentiation is blocked in anterior cingulated cortex (ACC) and lateral amygdala (LA) as determined by whole cell patch-clamp recordings,   (MGI Ref ID J:100197)
    • however, short-term synaptic plasticity and basal synaptic transmission are normal   (MGI Ref ID J:100197)
  • vision/eye phenotype
  • *normal* vision/eye phenotype
    • mice are wild-type for Pde6b and therefore sighted   (MGI Ref ID J:100197)

Fmr1tm1Cgr/Y

        FVB.129P2-Pde6b+ Tyrc-ch Fmr1tm1Cgr/J
  • behavior/neurological phenotype
  • abnormal locomotor activation
    • overall locomotor activity is increased as compared to wild-type   (MGI Ref ID J:171069)
    • mice on the FVB background are more active as compared to mice on the C57BL/6 background   (MGI Ref ID J:171069)
  • abnormal response to novelty
    • mice do not explore the novelty stimulus mouse in the social novelty preference test   (MGI Ref ID J:171069)
    • mice on the C57BL/6 background, but not the FVB background, exhibit an increased tendency to attack a juvenile stimulus mouse   (MGI Ref ID J:171069)
    • increased exploration in new environment
      • levels of spontaneous alternation and entries into maze arms are increased on the FVB background as compared to the C57BL/6 background   (MGI Ref ID J:171069)
      • however, exploration does not differ between mutant and wild-type   (MGI Ref ID J:171069)
  • abnormal social investigation
    • mice do not explore the novelty stimulus mouse in the social novelty preference test   (MGI Ref ID J:171069)
    • mice spend less time in non-social activities; the effect is more pronounced on the FVB background as compared to C57BL/6   (MGI Ref ID J:171069)
    • mice spend less time in affiliative behaviors; the effect is more pronounced on the C57BL/6 background as compared to FVB   (MGI Ref ID J:171069)
  • abnormal vocalization
    • mice exhibit an increase in the number of vocalization and duration on FVB background as compared to the C57BL/6 background   (MGI Ref ID J:171069)
  • decreased startle reflex
    • startle response is less prominent with increased pulse intensity as compared to wild-type   (MGI Ref ID J:171069)
    • mice on the C57BL/6 background exhibit stronger responses to 100 dBa pulse and lower responses to the 120 dBa pulse as compared to FVB   (MGI Ref ID J:171069)
  • nervous system phenotype
  • increased prepulse inhibition
    • magnitude of prepulse inhibition is enhanced although the effect is more prominent on the C57BL/6 background as compared to the FVB background   (MGI Ref ID J:171069)

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Fmr1tm1Cgr/Fmr1tm1Cgr

        involves: 129P2/OlaHsd * C57BL/6J
  • behavior/neurological phenotype
  • hyperactivity
    • mutants show significantly more crossings through three infrared beams in an empty cage over 40 min   (MGI Ref ID J:19220)
  • increased exploration in new environment
    • mutants exhibit more exploratory behavior than controls, displaying more line crossings in the lit compartment   (MGI Ref ID J:19220)

Fmr1tm1Cgr/Fmr1tm1Cgr

        involves: 129P2/OlaHsd
  • behavior/neurological phenotype
  • increased startle reflex
    • in all eye blink conditioning training session the percentage and peak amplitudes of the startle responses were higher   (MGI Ref ID J:101021)
  • nervous system phenotype
  • abnormal Purkinje cell morphology
    • the percentage of single climbing fiber innervation is increased, the length of spine heads and necks is increased, and spines are more irregular   (MGI Ref ID J:101021)
  • abnormal dentate gyrus morphology
    • increased volume of the dentate gyrus (DG) in mutant mice   (MGI Ref ID J:159211)
  • abnormal long term depression
    • induction of long term depression in Purkinje cells is significantly enhanced when stimulating parallel fibers   (MGI Ref ID J:101021)
  • abnormal neuron differentiation
    • 60.4% decrease in neuronal differentiation compared with wild-type isolated adult neural progenitor/stem cells (aNPCs)   (MGI Ref ID J:159211)
    • 74.9% increase in astrocyte differentiation compared with wild-type isolated adult neural progenitor/stem cells (aNPCs)   (MGI Ref ID J:159211)
    • exogenously expressed wild-type gene, but not mutant (I304N) rescues both the neuronal and the astrocyte differentiation deficits in homozygous mutant cells   (MGI Ref ID J:159211)
    • reduced (10.4% ) neuronal differentiation but greater (75.7%) glial differentiation in aNPCs residing in the DG compared with wild-type mice   (MGI Ref ID J:159211)
  • abnormal neuronal precursor proliferation
    • increased proliferation of isolated aNPCs from both the forebrain and DG of adult homozygous mice   (MGI Ref ID J:159211)
    • 11% more cells in mitotic (G2/M) phase compared with wild-type controls   (MGI Ref ID J:159211)
    • increased proliferation of both stem and progenitor cells in the DG and subventricular zone of mutant mice   (MGI Ref ID J:159211)
    • normal proliferation of astrocytes in the DG of mutant mice   (MGI Ref ID J:159211)
  • cellular phenotype
  • abnormal neuron differentiation
    • 60.4% decrease in neuronal differentiation compared with wild-type isolated adult neural progenitor/stem cells (aNPCs)   (MGI Ref ID J:159211)
    • 74.9% increase in astrocyte differentiation compared with wild-type isolated adult neural progenitor/stem cells (aNPCs)   (MGI Ref ID J:159211)
    • exogenously expressed wild-type gene, but not mutant (I304N) rescues both the neuronal and the astrocyte differentiation deficits in homozygous mutant cells   (MGI Ref ID J:159211)
    • reduced (10.4% ) neuronal differentiation but greater (75.7%) glial differentiation in aNPCs residing in the DG compared with wild-type mice   (MGI Ref ID J:159211)
  • abnormal neuronal precursor proliferation
    • increased proliferation of isolated aNPCs from both the forebrain and DG of adult homozygous mice   (MGI Ref ID J:159211)
    • 11% more cells in mitotic (G2/M) phase compared with wild-type controls   (MGI Ref ID J:159211)
    • increased proliferation of both stem and progenitor cells in the DG and subventricular zone of mutant mice   (MGI Ref ID J:159211)
    • normal proliferation of astrocytes in the DG of mutant mice   (MGI Ref ID J:159211)

Fmr1tm1Cgr/Fmr1tm1Cgr

        B6.129P2-Fmr1tm1Cgr
  • reproductive system phenotype
  • increased testis weight   (MGI Ref ID J:171292)
    • testes are significantly heavier than wild-type   (MGI Ref ID J:119166)
  • behavior/neurological phenotype
  • *normal* behavior/neurological phenotype
    • mice on the C57BL/6 background, but not the FVB/NJ background, location of hidden escape platform in water maze after 6 days of training   (MGI Ref ID J:119166)
    • abnormal object recognition memory
      • mice exhibit reduced novel object recognition compared with wild-type mice   (MGI Ref ID J:171292)
    • decreased aggression towards mice
      • mice exhibit less social dominance compared with wild-type mice   (MGI Ref ID J:171292)
    • hyperactivity   (MGI Ref ID J:171292)
    • impaired conditioned place preference behavior
      • for a scent-paired chamber   (MGI Ref ID J:171292)
    • increased vertical activity   (MGI Ref ID J:171292)
  • growth/size/body phenotype
  • increased body weight   (MGI Ref ID J:171292)
  • endocrine/exocrine gland phenotype
  • increased testis weight   (MGI Ref ID J:171292)
    • testes are significantly heavier than wild-type   (MGI Ref ID J:119166)

Fmr1tm1Cgr/Y

        involves: 129P2/OlaHsd * C57BL/6 * FVB
  • behavior/neurological phenotype
  • audiogenic seizures
    • 72% of mice have a seizure in response to the test tone   (MGI Ref ID J:127792)
  • impaired passive avoidance behavior
    • mice exhibit reduced latency to enter box 24 hours after initiation of inhibitory avoidance test as compared to wildtype (inhibitory avoidance extinction behavior)   (MGI Ref ID J:127792)
  • nervous system phenotype
  • abnormal dendrite morphology
    • dendritic spine density is increased in comparison to wildtype   (MGI Ref ID J:127792)
    • abnormal dendritic spine morphology
      • density of dendritic spines along apical dendrites of layer V pyramidal cells is increased   (MGI Ref ID J:70399)
      • dendritic spine length is increased   (MGI Ref ID J:70399)
      • mice have fewer short mushroom-shaped spines than wild-type   (MGI Ref ID J:70399)
      • elongated spines are more prevalent in mutant mice   (MGI Ref ID J:70399)
  • abnormal nerve fiber response
    • brief monocular deprivation results in substantial open-eye potentiation rather than the expected deprived-eye depression   (MGI Ref ID J:127792)
  • audiogenic seizures
    • 72% of mice have a seizure in response to the test tone   (MGI Ref ID J:127792)
  • growth/size/body phenotype
  • increased body weight
    • 10% increase in body weight is observed by postnatal day 26, but is similar to wildtype by day 45   (MGI Ref ID J:127792)
  • endocrine/exocrine gland phenotype
  • increased testis weight
    • increase in weight is only observed in adult (11-12 weeks)   (MGI Ref ID J:127792)
  • reproductive system phenotype
  • increased testis weight
    • increase in weight is only observed in adult (11-12 weeks)   (MGI Ref ID J:127792)

Fmr1tm1Cgr/Y

        involves: 129P2/OlaHsd * C57BL/6J
  • behavior/neurological phenotype
  • abnormal spatial learning
    • mutants do not seem to be impaired in the retrieval of spatial and nonspatial information in training and reversal trials once this information has been learned, but they are impaired in their acquisition of the reversal task   (MGI Ref ID J:19220)
  • hyperactivity
    • mutants show significantly more crossings through three infrared beams in an empty cage over 40 min   (MGI Ref ID J:19220)
  • increased exploration in new environment
    • mutants exhibit more exploratory behavior than controls, displaying more line crossings in the lit compartment   (MGI Ref ID J:19220)
  • endocrine/exocrine gland phenotype
  • enlarged testis
    • macroorchidism develops over time   (MGI Ref ID J:19220)
    • increased testis weight
      • weight of testis increases over time, however no structural abnormalities are observed   (MGI Ref ID J:19220)
  • reproductive system phenotype
  • enlarged testis
    • macroorchidism develops over time   (MGI Ref ID J:19220)
    • increased testis weight
      • weight of testis increases over time, however no structural abnormalities are observed   (MGI Ref ID J:19220)

Fmr1tm1Cgr/Y

        involves: 129P2/OlaHsd
  • behavior/neurological phenotype
  • abnormal eye blink conditioning behavior
    • the percentage of conditioned responses was reduced in the 2nd - 4th training sessions and peak amplitude and peak velocity were reduced in the 3rd and 4th training sessions   (MGI Ref ID J:101021)
  • abnormal spatial learning
    • in the reversal trials of the Morris water maze, mutants take more time to escape to the platform than controls   (MGI Ref ID J:34449)
  • increased startle reflex
    • in all eye blink conditioning training session the percentage and peak amplitudes of the startle responses were higher   (MGI Ref ID J:101021)
  • endocrine/exocrine gland phenotype
  • enlarged testis
    • progressive enlargement resulting in testes that were 34% bigger than those of wild-type by 168-170 days of age   (MGI Ref ID J:34449)
    • macroorchidism decreases somewhat after 168-170 days of age   (MGI Ref ID J:34449)
    • increased testis weight   (MGI Ref ID J:34449)
  • reproductive system phenotype
  • enlarged testis
    • progressive enlargement resulting in testes that were 34% bigger than those of wild-type by 168-170 days of age   (MGI Ref ID J:34449)
    • macroorchidism decreases somewhat after 168-170 days of age   (MGI Ref ID J:34449)
    • increased testis weight   (MGI Ref ID J:34449)
  • nervous system phenotype
  • abnormal Purkinje cell morphology
    • the percentage of single climbing fiber innervation is increased, the length of spine heads and necks is increased, and spines are more irregular   (MGI Ref ID J:101021)
  • abnormal long term depression
    • induction of long term depression in Purkinje cells is significantly enhanced when stimulating parallel fibers   (MGI Ref ID J:101021)

Fmr1tm1Cgr/Y

        involves: 129P2/OlaHsd * FVB
  • behavior/neurological phenotype
  • convulsive seizures
    • some mice exhibit bilateral motor seizures that range from head twitching to forelimb and hindlimb clonus, loss of postural control and tonic twisting of head and torso   (MGI Ref ID J:113177)
    • tonic-clonic seizures
      • observed in some mice   (MGI Ref ID J:113177)
  • decreased anxiety-related response
    • total number of entries in arms of elevated plus maze was significantly decreased as compared to littermate control in one of the two cohorts tested   (MGI Ref ID J:151144)
  • impaired coordination
    • mice on the C57BL/6 background have longer latencies on the rotarod test than mice on the mixed FVB and 129P2 background, however, the difference between mutant and control on the mixed FVB and 129P2 background is not significant   (MGI Ref ID J:151144)
  • increased anxiety-related response
    • percentage of entries and percentage of time spent in arms of elevated plus maze is significantly increased as compared to Fmr1tm1Cgr mice on the C57BL/6 background, however, the difference between mutant and control on the mixed FVB and 129P2 background is not significant   (MGI Ref ID J:151144)
    • distance traveled in open field test is increased as compared to control   (MGI Ref ID J:151144)
  • increased exploration in new environment
    • distance traveled in open field test is increased as compared to control   (MGI Ref ID J:151144)
  • social withdrawal
    • mice spend more time in an empty cage than in a cage with a strange mouse in sociability choice test   (MGI Ref ID J:151144)
  • growth/size/body phenotype
  • increased body weight
    • body weight was significantly increased in one of the two cohorts tested   (MGI Ref ID J:151144)
  • nervous system phenotype
  • abnormal hippocampal mossy fiber morphology
    • density of Timm granules (Timm staining identifies neural elements that contain heavy metals) in zinc-rich mossy fiber terminals is increased in inner molecular layer and close to the hilus in the stratum oriens   (MGI Ref ID J:113177)
  • convulsive seizures
    • some mice exhibit bilateral motor seizures that range from head twitching to forelimb and hindlimb clonus, loss of postural control and tonic twisting of head and torso   (MGI Ref ID J:113177)
    • tonic-clonic seizures
      • observed in some mice   (MGI Ref ID J:113177)
  • vision/eye phenotype
  • *normal* vision/eye phenotype   (MGI Ref ID J:151144)
    • mice are wild-type at the Pde6b locus and are therefore not blind   (MGI Ref ID J:113177)

Fmr1tm1Cgr/Y

        B6.129P2(FVB)-Fmr1tm1Cgr
  • behavior/neurological phenotype
  • *normal* behavior/neurological phenotype
    • mice on the C57BL/6 background have longer latencies on the rotarod test than mice on the mixed FVB and 129P2 background, however, the difference between mutant and control on the C57BL/6 background is not significant   (MGI Ref ID J:151144)
    • decreased anxiety-related response
      • percentage of entries and percentage of time spent in arms of elevated plus maze is significantly decreased as compared to Fmr1tm1Cgr mice on the mixed FVB and 129P2 background, however, the difference between mutant and control on the C57BL/6 background is not significant   (MGI Ref ID J:151144)
    • social withdrawal
      • mice spend more time in an empty cage than in a cage with a strange mouse in the sociability choice test in one of two cohorts   (MGI Ref ID J:151144)

Fmr1tm1Cgr/Y

        FVB.129P2(B6)-Fmr1tm1Cgr/J
  • behavior/neurological phenotype
  • decreased startle reflex
    • startle response development is normal (at 118 dB) until 3-4 weeks of age, but does not increase as compared to controls   (MGI Ref ID J:113024)

Fmr1tm1Cgr/Y

        either: FVB.129P2(B6)-Fmr1tm1Cgr/J or FVB;129P2(B6)-Fmr1tm1Cgr/J
  • behavior/neurological phenotype
  • audiogenic seizures
    • loud (115 dB) sound results in seizures   (MGI Ref ID J:85912)
    • seizures begin within 20-30 seconds and are characterized by wild running, erratic leaping, clonic convulsions and progress to tonic hindlimb extension, respiratory arrest and death   (MGI Ref ID J:85912)
    • seizures are age dependent and are not fully penetrant   (MGI Ref ID J:85912)
    • no seizures occur before 10 weeks of age   (MGI Ref ID J:85912)
    • 57% of mice exhibit seizures between 10-12 weeks; 70% of mice exhibit seizures between 20-34 weeks of age   (MGI Ref ID J:85912)
  • decreased startle reflex   (MGI Ref ID J:85912)
  • nervous system phenotype
  • audiogenic seizures
    • loud (115 dB) sound results in seizures   (MGI Ref ID J:85912)
    • seizures begin within 20-30 seconds and are characterized by wild running, erratic leaping, clonic convulsions and progress to tonic hindlimb extension, respiratory arrest and death   (MGI Ref ID J:85912)
    • seizures are age dependent and are not fully penetrant   (MGI Ref ID J:85912)
    • no seizures occur before 10 weeks of age   (MGI Ref ID J:85912)
    • 57% of mice exhibit seizures between 10-12 weeks; 70% of mice exhibit seizures between 20-34 weeks of age   (MGI Ref ID J:85912)
  • increased prepulse inhibition
    • both a low (75 dB) and higher (85 dB) prepulse inhibits startle response more efficiently as compared to wild-type   (MGI Ref ID J:85912)

Fmr1tm1Cgr/Y

        B6.129P2-Fmr1tm1Cgr/Nwu
  • behavior/neurological phenotype
  • *normal* behavior/neurological phenotype
    • levels of spontaneous alternation and entries into maze arms are increased on the FVB background as compared to the C57BL/6 background, however, exploration does not differ between mutant and wild-type   (MGI Ref ID J:171069)
    • mice exhibit an increase in the number of vocalization and duration on FVB background as compared to the C57BL/6 background   (MGI Ref ID J:171069)
    • abnormal locomotor activation
      • overall locomotor activity is increased as compared to wild-type   (MGI Ref ID J:171069)
      • mice on the FVB background are more active as compared to the C57BL/6 background   (MGI Ref ID J:171069)
    • abnormal response to novelty
      • mice do not preferentially explore the novelty stimulus mouse as compared to the familiar mouse in the social novelty preference test   (MGI Ref ID J:171069)
    • abnormal social investigation
      • mice do not preferentially explore the novelty stimulus mouse as compared to the familiar mouse in the social novelty preference test   (MGI Ref ID J:171069)
      • mice spend less time in non-social activities; the effect is more pronounced on the FVB background as compared to C57BL/6   (MGI Ref ID J:171069)
      • mice spend less time in affiliative behaviors; the effect is more pronounced on the C57BL/6 background as compared to FVB   (MGI Ref ID J:171069)
    • decreased startle reflex
      • startle response is less prominent with increased pulse intensity as compared to wild-type   (MGI Ref ID J:171069)
      • mice on the C57BL/6 background exhibit stronger responses to 100 dBa pulse and lower responses to the 120 dBa pulse as compared to FVB   (MGI Ref ID J:171069)
    • increased aggression towards mice
      • mice on the C57BL/6 background, but not the FVB background, exhibit an increased tendency to attack a juvenile stimulus mouse   (MGI Ref ID J:171069)
    • increased grooming behavior
      • mice spend more time self-grooming; the effect is more pronounced on the C57BL/6 background as compared to the FVB background   (MGI Ref ID J:171069)
  • nervous system phenotype
  • increased prepulse inhibition
    • magnitude of prepulse inhibition is enhanced although the effect is more prominent on the C57BL/6 background as compared to the FVB background   (MGI Ref ID J:171069)

Fmr1tm1Cgr/Y

        involves: 129P2/OlaHsd * FVB/NJ
  • behavior/neurological phenotype
  • abnormal spatial learning
    • mice do not learn location of hidden escape platform in water maze after 6 days of training   (MGI Ref ID J:119166)
    • in contrast, mice on the C57BL/6 background learned platform location and performed similar to wild-type   (MGI Ref ID J:119166)
  • endocrine/exocrine gland phenotype
  • increased testis weight
    • testes are significantly heavier than wild-type   (MGI Ref ID J:119166)
  • vision/eye phenotype
  • *normal* vision/eye phenotype
    • mice have at least one wild-type allele of Pde6b and therefore lack vison impairments commonly seen in FVB mice   (MGI Ref ID J:119166)
  • reproductive system phenotype
  • increased testis weight
    • testes are significantly heavier than wild-type   (MGI Ref ID J:119166)
View Research Applications

Research Applications
This mouse can be used to support research in many areas including:

Fmr1tm1Cgr related

Developmental Biology Research
Internal/Organ Defects
      multiple

Neurobiology Research
Behavioral and Learning Defects
Fragile X Mental Retardation Syndrome

Pde6b+ related

Sensorineural Research
Retinal Degeneration
      wild-type

Tyrc-ch related

Dermatology Research
Color and White Spotting Defects

Developmental Biology Research
Neurodevelopmental Defects
Skeletal Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Fmr1tm1Cgr
Allele Name targeted mutation 1, Ben Oostra
Allele Type Targeted (Null/Knockout)
Common Name(s) FMRP KO; Fmr1 KO; Fmr1tm4Cgr; FraX; fmr-tm1Cgr;
Mutation Made ByDr. Ben Oostra,   Erasmus University
Strain of Origin129P2/OlaHsd
ES Cell Line NameE14
ES Cell Line Strain129P2/OlaHsd
Gene Symbol and Name Fmr1, fragile X mental retardation syndrome 1
Chromosome X
Gene Common Name(s) FMRP; FRAXA; Fmr-1; POF; POF1;
General Note Genbank: AF179463 and AF170530
Molecular Note A neomycin resistance gene was inserted into exon 5. RT-PCR analysis on testis RNA derived from hemizygous male mice demonstrated that no detectable transcript was produced from this allele, and western blot analysis on extracts of testes, liver, kidneyand brain of hemizygous male mice confirmed that no stable encoded protein was made. [MGI Ref ID J:19220]
 
Allele Symbol Pde6b+
Allele Name wild type
Allele Type Not Applicable
Mutation Made By Frank Kooy,   University of Antwerp
Gene Symbol and Name Pde6b, phosphodiesterase 6B, cGMP, rod receptor, beta polypeptide
Chromosome 5
Gene Common Name(s) CSNB3; CSNBAD2; PDEB; Pdeb; RP40; nmf137; phosphodiesterase, cGMP, rod receptor, beta polypeptide; r; rd; rd-1; rd1; rd10; retinal degeneration; retinal degeneration 1; retinal degeneration 10;
 
Allele Symbol Tyrc-ch
Allele Name chinchilla
Allele Type Spontaneous
Common Name(s) cch; cr;
Strain of Originfancier's stock
Gene Symbol and Name Tyr, tyrosinase
Chromosome 7
Gene Common Name(s) ATN; C; CMM8; OCA1; OCA1A; OCAIA; SHEP3; albino; c; skc35; skin/coat color 35;
Molecular Note The mutation in the chinchilla allele was found to be a G to A point mutation that results in an amino acid change at position 464 from alanine to threonine. [MGI Ref ID J:19279]

Genotyping

Genotyping Information

Genotyping Protocols

Fmr1tm1Cgr,

Separated MCA


Fmr1tm1Cgr, Melt Curve Analysis
Fmr1tm1Cgr, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Bakker CE; Verheij C; Willemsen R; Vanderhelm R; Oerlemans F; Vermey M; Bygrave A; Hoogeveen AT; Oostra BA; Reyniers E; Deboulle K; Dhooge R; Cras P; Vanvelzen D; Nagels G; Martin JJ; Dedyn PP; Darby JK; Willems PJ; The Dutch-Belgium Fragile X Consortium. 1994. Fmr1 knockout mice: a model to study fragile x mental retardation. The Dutch-Belgium Fragile X Consortium Cell 78(1):23-33. [PubMed: 8033209]  [MGI Ref ID J:19220]

Additional References

Irwin SA; Idupulapati M; Gilbert ME; Harris JB; Chakravarti AB; Rogers EJ; Crisostomo RA; Larsen BP; Mehta A; Alcantara CJ; Patel B; Swain RA; Weiler IJ; Oostra BA; Greenough WT. 2002. Dendritic spine and dendritic field characteristics of layer V pyramidal neurons in the visual cortex of fragile-X knockout mice. Am J Med Genet 111(2):140-6. [PubMed: 12210340]  [MGI Ref ID J:78149]

Fmr1tm1Cgr related

Achuta VS; Rezov V; Uutela M; Louhivuori V; Louhivuori L; Castren ML. 2014. Tissue plasminogen activator contributes to alterations of neuronal migration and activity-dependent responses in fragile X mice. J Neurosci 34(5):1916-23. [PubMed: 24478370]  [MGI Ref ID J:206954]

Alpatov R; Lesch BJ; Nakamoto-Kinoshita M; Blanco A; Chen S; Stutzer A; Armache KJ; Simon MD; Xu C; Ali M; Murn J; Prisic S; Kutateladze TG; Vakoc CR; Min J; Kingston RE; Fischle W; Warren ST; Page DC; Shi Y. 2014. A chromatin-dependent role of the fragile X mental retardation protein FMRP in the DNA damage response. Cell 157(4):869-81. [PubMed: 24813610]  [MGI Ref ID J:214397]

Antar LN; Li C; Zhang H; Carroll RC; Bassell GJ. 2006. Local functions for FMRP in axon growth cone motility and activity-dependent regulation of filopodia and spine synapses. Mol Cell Neurosci 32(1-2):37-48. [PubMed: 16631377]  [MGI Ref ID J:111946]

Ascano M Jr; Mukherjee N; Bandaru P; Miller JB; Nusbaum JD; Corcoran DL; Langlois C; Munschauer M; Dewell S; Hafner M; Williams Z; Ohler U; Tuschl T. 2012. FMRP targets distinct mRNA sequence elements to regulate protein expression. Nature 492(7429):382-6. [PubMed: 23235829]  [MGI Ref ID J:194411]

Aschrafi A; Cunningham BA; Edelman GM; Vanderklish PW. 2005. The fragile X mental retardation protein and group I metabotropic glutamate receptors regulate levels of mRNA granules in brain. Proc Natl Acad Sci U S A 102(6):2180-5. [PubMed: 15684045]  [MGI Ref ID J:96472]

Auerbach BD; Osterweil EK; Bear MF. 2011. Mutations causing syndromic autism define an axis of synaptic pathophysiology. Nature 480(7375):63-8. [PubMed: 22113615]  [MGI Ref ID J:178293]

Baker KB; Wray SP; Ritter R; Mason S; Lanthorn TH; Savelieva KV. 2010. Male and female Fmr1 knockout mice on C57 albino background exhibit spatial learning and memory impairments. Genes Brain Behav 9(6):562-74. [PubMed: 20398059]  [MGI Ref ID J:175059]

Bakker CE; de Diego Otero Y; Bontekoe C; Raghoe P; Luteijn T; Hoogeveen AT; Oostra BA; Willemsen R. 2000. Immunocytochemical and biochemical characterization of FMRP, FXR1P, and FXR2P in the mouse Exp Cell Res 258(1):162-70. [PubMed: 10912798]  [MGI Ref ID J:63269]

Bilousova TV; Dansie L; Ngo M; Aye J; Charles JR; Ethell DW; Ethell IM. 2009. Minocycline promotes dendritic spine maturation and improves behavioural performance in the fragile X mouse model. J Med Genet 46(2):94-102. [PubMed: 18835858]  [MGI Ref ID J:200885]

Bongmba OY; Martinez LA; Elhardt ME; Butler K; Tejada-Simon MV. 2011. Modulation of dendritic spines and synaptic function by Rac1: A possible link to Fragile X syndrome pathology. Brain Res 1399:79-95. [PubMed: 21645877]  [MGI Ref ID J:174070]

Braun K; Segal M. 2000. FMRP involvement in formation of synapses among cultured hippocampal neurons. Cereb Cortex 10(10):1045-52. [PubMed: 11007555]  [MGI Ref ID J:102340]

Brennan FX; Albeck DS; Paylor R. 2006. Fmr1 knockout mice are impaired in a leverpress escape/avoidance task. Genes Brain Behav 5(6):467-71. [PubMed: 16923151]  [MGI Ref ID J:123642]

Brown MR; Kronengold J; Gazula VR; Chen Y; Strumbos JG; Sigworth FJ; Navaratnam D; Kaczmarek LK. 2010. Fragile X mental retardation protein controls gating of the sodium-activated potassium channel Slack. Nat Neurosci 13(7):819-21. [PubMed: 20512134]  [MGI Ref ID J:161627]

Bureau I; Shepherd GM; Svoboda K. 2008. Circuit and plasticity defects in the developing somatosensory cortex of FMR1 knock-out mice. J Neurosci 28(20):5178-88. [PubMed: 18480274]  [MGI Ref ID J:136321]

Busquets-Garcia A; Gomis-Gonzalez M; Guegan T; Agustin-Pavon C; Pastor A; Mato S; Perez-Samartin A; Matute C; de la Torre R; Dierssen M; Maldonado R; Ozaita A. 2013. Targeting the endocannabinoid system in the treatment of fragile X syndrome. Nat Med 19(5):603-7. [PubMed: 23542787]  [MGI Ref ID J:198359]

Centonze D; Rossi S; Mercaldo V; Napoli I; Ciotti MT; De Chiara V; Musella A; Prosperetti C; Calabresi P; Bernardi G; Bagni C. 2008. Abnormal striatal GABA transmission in the mouse model for the fragile X syndrome. Biol Psychiatry 63(10):963-73. [PubMed: 18028882]  [MGI Ref ID J:139619]

Centonze D; Rossi S; Napoli I; Mercaldo V; Lacoux C; Ferrari F; Ciotti MT; De Chiara V; Prosperetti C; Maccarrone M; Fezza F; Calabresi P; Bernardi G; Bagni C. 2007. The brain cytoplasmic RNA BC1 regulates dopamine D2 receptor-mediated transmission in the striatum. J Neurosci 27(33):8885-92. [PubMed: 17699670]  [MGI Ref ID J:145252]

Chen L; Toth M. 2001. Fragile X mice develop sensory hyperreactivity to auditory stimuli. Neuroscience 103(4):1043-50. [PubMed: 11301211]  [MGI Ref ID J:85912]

Chen LY; Rex CS; Babayan AH; Kramar EA; Lynch G; Gall CM; Lauterborn JC. 2010. Physiological activation of synaptic Rac>PAK (p-21 activated kinase) signaling is defective in a mouse model of fragile X syndrome. J Neurosci 30(33):10977-84. [PubMed: 20720104]  [MGI Ref ID J:163307]

Chuang SC; Zhao W; Bauchwitz R; Yan Q; Bianchi R; Wong RK. 2005. Prolonged epileptiform discharges induced by altered group I metabotropic glutamate receptor-mediated synaptic responses in hippocampal slices of a fragile X mouse model. J Neurosci 25(35):8048-55. [PubMed: 16135762]  [MGI Ref ID J:100474]

Comery TA; Harris JB; Willems PJ; Oostra BA; Irwin SA; Weiler IJ; Greenough WT. 1997. Abnormal dendritic spines in fragile X knockout mice: maturation and pruning deficits. Proc Natl Acad Sci U S A 94(10):5401-4. [PubMed: 9144249]  [MGI Ref ID J:70399]

Cruz-Martin A; Crespo M; Portera-Cailliau C. 2010. Delayed stabilization of dendritic spines in fragile X mice. J Neurosci 30(23):7793-803. [PubMed: 20534828]  [MGI Ref ID J:160891]

D'Antuono M; Merlo D; Avoli M. 2003. Involvement of cholinergic and gabaergic systems in the fragile X knockout mice. Neuroscience 119(1):9-13. [PubMed: 12763063]  [MGI Ref ID J:126733]

D'Hooge R; Nagels G; Franck F; Bakker CE; Reyniers E; Storm K ; Kooy RF ; Oostra BA ; Willems PJ ; De Deyn PP. 1997. Mildly impaired water maze performance in male Fmr1 knockout mice. Neuroscience 76(2):367-76. [PubMed: 9015322]  [MGI Ref ID J:38436]

D'Hulst C; De Geest N; Reeve SP; Van Dam D; De Deyn PP; Hassan BA; Kooy RF. 2006. Decreased expression of the GABA(A) receptor in fragile X syndrome. Brain Res 1121(1):238-45. [PubMed: 17046729]  [MGI Ref ID J:115258]

D'Hulst C; Heulens I; Brouwer JR; Willemsen R; De Geest N; Reeve SP; De Deyn PP; Hassan BA; Kooy RF. 2009. Expression of the GABAergic system in animal models for fragile X syndrome and fragile X associated tremor/ataxia syndrome (FXTAS). Brain Res 1253:176-83. [PubMed: 19070606]  [MGI Ref ID J:147799]

Dahlhaus R; El-Husseini A. 2010. Altered neuroligin expression is involved in social deficits in a mouse model of the fragile X syndrome. Behav Brain Res 208(1):96-105. [PubMed: 19932134]  [MGI Ref ID J:157012]

Dansie LE; Phommahaxay K; Okusanya AG; Uwadia J; Huang M; Rotschafer SE; Razak KA; Ethell DW; Ethell IM. 2013. Long-lasting effects of minocycline on behavior in young but not adult Fragile X mice. Neuroscience 246:186-98. [PubMed: 23660195]  [MGI Ref ID J:201443]

Darnell JC; Van Driesche SJ; Zhang C; Hung KY; Mele A; Fraser CE; Stone EF; Chen C; Fak JJ; Chi SW; Licatalosi DD; Richter JD; Darnell RB. 2011. FMRP Stalls Ribosomal Translocation on mRNAs Linked to Synaptic Function and Autism. Cell 146(2):247-61. [PubMed: 21784246]  [MGI Ref ID J:174610]

Davidovic L; Navratil V; Bonaccorso CM; Catania MV; Bardoni B; Dumas ME. 2011. A metabolomic and systems biology perspective on the brain of the fragile X syndrome mouse model. Genome Res 21(12):2190-202. [PubMed: 21900387]  [MGI Ref ID J:183567]

De Rubeis S; Pasciuto E; Li KW; Fernandez E; Di Marino D; Buzzi A; Ostroff LE; Klann E; Zwartkruis FJ; Komiyama NH; Grant SG; Poujol C; Choquet D; Achsel T; Posthuma D; Smit AB; Bagni C. 2013. CYFIP1 Coordinates mRNA Translation and Cytoskeleton Remodeling to Ensure Proper Dendritic Spine Formation. Neuron 79(6):1169-82. [PubMed: 24050404]  [MGI Ref ID J:201819]

Deng PY; Rotman Z; Blundon JA; Cho Y; Cui J; Cavalli V; Zakharenko SS; Klyachko VA. 2013. FMRP regulates neurotransmitter release and synaptic information transmission by modulating action potential duration via BK channels. Neuron 77(4):696-711. [PubMed: 23439122]  [MGI Ref ID J:197827]

Deng PY; Sojka D; Klyachko VA. 2011. Abnormal presynaptic short-term plasticity and information processing in a mouse model of fragile x syndrome. J Neurosci 31(30):10971-82. [PubMed: 21795546]  [MGI Ref ID J:174605]

Denman RB; Xie W; Merz G; Sung YJ. 2014. GABAAergic stimulation modulates intracellular protein arginine methylation. Neurosci Lett 572:38-43. [PubMed: 24793772]  [MGI Ref ID J:214002]

Desai NS; Casimiro TM; Gruber SM; Vanderklish PW. 2006. Early postnatal plasticity in neocortex of Fmr1 knockout mice. J Neurophysiol 96(4):1734-45. [PubMed: 16823030]  [MGI Ref ID J:135706]

Dickson PE; Corkill B; McKimm E; Miller MM; Calton MA; Goldowitz D; Blaha CD; Mittleman G. 2013. Effects of stimulus salience on touchscreen serial reversal learning in a mouse model of fragile X syndrome. Behav Brain Res 252:126-35. [PubMed: 23747611]  [MGI Ref ID J:202112]

Dictenberg JB; Swanger SA; Antar LN; Singer RH; Bassell GJ. 2008. A direct role for FMRP in activity-dependent dendritic mRNA transport links filopodial-spine morphogenesis to fragile X syndrome. Dev Cell 14(6):926-39. [PubMed: 18539120]  [MGI Ref ID J:137197]

Dobkin C; Rabe A; Dumas R; El Idrissi A; Haubenstock H; Brown WT. 2000. Fmr1 knockout mouse has a distinctive strain-specific learning impairment. Neuroscience 100(2):423-9. [PubMed: 11008180]  [MGI Ref ID J:119166]

Dolan BM; Duron SG; Campbell DA; Vollrath B; Rao BS; Ko HY; Lin GG; Govindarajan A; Choi SY; Tonegawa S. 2013. Rescue of fragile X syndrome phenotypes in Fmr1 KO mice by the small-molecule PAK inhibitor FRAX486. Proc Natl Acad Sci U S A 110(14):5671-6. [PubMed: 23509247]  [MGI Ref ID J:194236]

Dolen G; Osterweil E; Rao BS; Smith GB; Auerbach BD; Chattarji S; Bear MF. 2007. Correction of Fragile X Syndrome in Mice. Neuron 56(6):955-962. [PubMed: 18093519]  [MGI Ref ID J:127792]

Dury AY; El Fatimy R; Tremblay S; Rose TM; Cote J; De Koninck P; Khandjian EW. 2013. Nuclear Fragile X Mental Retardation Protein is localized to Cajal bodies. PLoS Genet 9(10):e1003890. [PubMed: 24204304]  [MGI Ref ID J:204185]

El Idrissi A; Ding XH; Scalia J; Trenkner E; Brown WT; Dobkin C. 2005. Decreased GABA(A) receptor expression in the seizure-prone fragile X mouse. Neurosci Lett 377(3):141-6. [PubMed: 15755515]  [MGI Ref ID J:104931]

Errijgers V; Fransen E; D'Hooge R; De Deyn PP; Kooy RF. 2008. Effect of genetic background on acoustic startle response in fragile X knockout mice. Genet Res 90(4):341-5. [PubMed: 18840308]  [MGI Ref ID J:144419]

Fisch GS; Hao HK; Bakker C; Oostra BA. 1999. Learning and memory in the FMR1 knockout mouse. Am J Med Genet 84(3):277-82. [PubMed: 10331607]  [MGI Ref ID J:54509]

Fish EW; Krouse MC; Stringfield SJ; Diberto JF; Robinson JE; Malanga CJ. 2013. Changes in sensitivity of reward and motor behavior to dopaminergic, glutamatergic, and cholinergic drugs in a mouse model of fragile X syndrome. PLoS One 8(10):e77896. [PubMed: 24205018]  [MGI Ref ID J:209243]

Frankland PW; Wang Y; Rosner B; Shimizu T; Balleine BW; Dykens EM; Ornitz EM; Silva AJ. 2004. Sensorimotor gating abnormalities in young males with fragile X syndrome and Fmr1-knockout mice. Mol Psychiatry 9(4):417-25. [PubMed: 14981523]  [MGI Ref ID J:102061]

Galvan AM; Galvez R. 2012. Neocortical vasculature abnormalities in the Fragile X mental retardation syndrome. Brain Res 1471:155-61. [PubMed: 22796598]  [MGI Ref ID J:192392]

Galvez R; Gopal AR; Greenough WT. 2003. Somatosensory cortical barrel dendritic abnormalities in a mouse model of the fragile X mental retardation syndrome. Brain Res 971(1):83-9. [PubMed: 12691840]  [MGI Ref ID J:83158]

Galvez R; Smith RL; Greenough WT. 2005. Olfactory bulb mitral cell dendritic pruning abnormalities in a mouse model of the Fragile-X mental retardation syndrome: further support for FMRP's involvement in dendritic development. Brain Res Dev Brain Res 157(2):214-6. [PubMed: 15878626]  [MGI Ref ID J:104546]

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Seese RR; Babayan AH; Katz AM; Cox CD; Lauterborn JC; Lynch G; Gall CM. 2012. LTP induction translocates cortactin at distant synapses in wild-type but not FMR1 knock-out mice. J Neurosci 32(21):7403-13. [PubMed: 22623686]  [MGI Ref ID J:184974]

Segal M; Kreher U; Greenberger V; Braun K. 2003. Is fragile X mental retardation protein involved in activity-induced plasticity of dendritic spines? Brain Res 972(1-2):9-15. [PubMed: 12711073]  [MGI Ref ID J:107795]

Selby L; Zhang C; Sun QQ. 2007. Major defects in neocortical GABAergic inhibitory circuits in mice lacking the fragile X mental retardation protein. Neurosci Lett 412(3):227-32. [PubMed: 17197085]  [MGI Ref ID J:119086]

Sharma A; Hoeffer CA; Takayasu Y; Miyawaki T; McBride SM; Klann E; Zukin RS. 2010. Dysregulation of mTOR signaling in fragile X syndrome. J Neurosci 30(2):694-702. [PubMed: 20071534]  [MGI Ref ID J:157708]

Shi D; Xu S; Waddell J; Scafidi S; Roys S; Gullapalli RP; McKenna MC. 2012. Longitudinal in vivo developmental changes of metabolites in the hippocampus of Fmr1 knockout mice. J Neurochem 123(6):971-81. [PubMed: 23046047]  [MGI Ref ID J:191000]

Slegtenhorst-Eegdeman KE; de Rooij DG; Verhoef-Post M; van de Kant HJ; Bakker CE; Oostra BA; Grootegoed JA; Themmen AP. 1998. Macroorchidism in FMR1 knockout mice is caused by increased Sertoli cell proliferation during testicular development. Endocrinology 139(1):156-62. [PubMed: 9421410]  [MGI Ref ID J:44826]

Spencer CM; Alekseyenko O; Serysheva E; Yuva-Paylor LA; Paylor R. 2005. Altered anxiety-related and social behaviors in the Fmr1 knockout mouse model of fragile X syndrome. Genes Brain Behav 4(7):420-30. [PubMed: 16176388]  [MGI Ref ID J:114353]

Spencer CM; Serysheva E; Yuva-Paylor LA; Oostra BA; Nelson DL; Paylor R. 2006. Exaggerated behavioral phenotypes in Fmr1/Fxr2 double knockout mice reveal a functional genetic interaction between Fragile X-related proteins. Hum Mol Genet 15(12):1984-94. [PubMed: 16675531]  [MGI Ref ID J:112066]

Steward O; Bakker CE; Willems PJ; Oostra BA. 1998. No evidence for disruption of normal patterns of mRNA localization in dendrites or dendritic transport of recently synthesized mRNA in FMR1 knockout mice, a model for human fragile-X mental retardation syndrome. Neuroreport 9(3):477-81. [PubMed: 9512393]  [MGI Ref ID J:103598]

Straiker A; Min KT; Mackie K. 2013. Fmr1 deletion enhances and ultimately desensitizes CB1 signaling in autaptic hippocampal neurons. Neurobiol Dis 56:1-5. [PubMed: 23578490]  [MGI Ref ID J:197902]

Strumbos JG; Brown MR; Kronengold J; Polley DB; Kaczmarek LK. 2010. Fragile X mental retardation protein is required for rapid experience-dependent regulation of the potassium channel Kv3.1b. J Neurosci 30(31):10263-71. [PubMed: 20685971]  [MGI Ref ID J:162850]

Suvrathan A; Hoeffer CA; Wong H; Klann E; Chattarji S. 2010. Characterization and reversal of synaptic defects in the amygdala in a mouse model of fragile X syndrome. Proc Natl Acad Sci U S A 107(25):11591-6. [PubMed: 20534533]  [MGI Ref ID J:161384]

Tervonen T; Akerman K; Oostra BA; Castren M. 2005. Rgs4 mRNA expression is decreased in the brain of Fmr1 knockout mouse. Brain Res Mol Brain Res 133(1):162-5. [PubMed: 15661377]  [MGI Ref ID J:95566]

Tervonen TA; Louhivuori V; Sun X; Hokkanen ME; Kratochwil CF; Zebryk P; Castren E; Castren ML. 2009. Aberrant differentiation of glutamatergic cells in neocortex of mouse model for fragile X syndrome. Neurobiol Dis 33(2):250-9. [PubMed: 19056494]  [MGI Ref ID J:144369]

Testa-Silva G; Loebel A; Giugliano M; de Kock CP; Mansvelder HD; Meredith RM. 2012. Hyperconnectivity and slow synapses during early development of medial prefrontal cortex in a mouse model for mental retardation and autism. Cereb Cortex 22(6):1333-42. [PubMed: 21856714]  [MGI Ref ID J:198110]

Till SM; Wijetunge LS; Seidel VG; Harlow E; Wright AK; Bagni C; Contractor A; Gillingwater TH; Kind PC. 2012. Altered maturation of the primary somatosensory cortex in a mouse model of fragile X syndrome. Hum Mol Genet 21(10):2143-56. [PubMed: 22328088]  [MGI Ref ID J:183805]

Tsai NP; Wilkerson JR; Guo W; Maksimova MA; DeMartino GN; Cowan CW; Huber KM. 2012. Multiple autism-linked genes mediate synapse elimination via proteasomal degradation of a synaptic scaffold PSD-95. Cell 151(7):1581-94. [PubMed: 23260144]  [MGI Ref ID J:193326]

Udagawa T; Farny NG; Jakovcevski M; Kaphzan H; Alarcon JM; Anilkumar S; Ivshina M; Hurt JA; Nagaoka K; Nalavadi VC; Lorenz LJ; Bassell GJ; Akbarian S; Chattarji S; Klann E; Richter JD. 2013. Genetic and acute CPEB1 depletion ameliorate fragile X pathophysiology. Nat Med 19(11):1473-7. [PubMed: 24141422]  [MGI Ref ID J:203765]

Uutela M; Lindholm J; Louhivuori V; Wei H; Louhivuori LM; Pertovaara A; Akerman K; Castren E; Castren ML. 2012. Reduction of BDNF expression in Fmr1 knockout mice worsens cognitive deficits but improves hyperactivity and sensorimotor deficits. Genes Brain Behav 11(5):513-23. [PubMed: 22435671]  [MGI Ref ID J:198036]

Van Dam D; D'Hooge R; Hauben E; Reyniers E; Gantois I; Bakker CE; Oostra BA; Kooy RF; De Deyn PP. 2000. Spatial learning, contextual fear conditioning and conditioned emotional response in Fmr1 knockout mice. Behav Brain Res 117(1-2):127-36. [PubMed: 11099766]  [MGI Ref ID J:96655]

Van Dam D; Errijgers V; Kooy RF; Willemsen R; Mientjes E; Oostra BA; De Deyn PP. 2005. Cognitive decline, neuromotor and behavioural disturbances in a mouse model for fragile-X-associated tremor/ataxia syndrome (FXTAS). Behav Brain Res 162(2):233-9. [PubMed: 15876460]  [MGI Ref ID J:104445]

Veeraragavan S; Graham D; Bui N; Yuva-Paylor LA; Wess J; Paylor R. 2012. Genetic reduction of muscarinic M4 receptor modulates analgesic response and acoustic startle response in a mouse model of fragile X syndrome (FXS). Behav Brain Res 228(1):1-8. [PubMed: 22123412]  [MGI Ref ID J:185107]

Vislay RL; Martin BS; Olmos-Serrano JL; Kratovac S; Nelson DL; Corbin JG; Huntsman MM. 2013. Homeostatic responses fail to correct defective amygdala inhibitory circuit maturation in fragile X syndrome. J Neurosci 33(17):7548-58. [PubMed: 23616559]  [MGI Ref ID J:197033]

Wang H; Wu LJ; Kim SS; Lee FJ; Gong B; Toyoda H; Ren M; Shang YZ; Xu H; Liu F; Zhao MG; Zhuo M. 2008. FMRP acts as a key messenger for dopamine modulation in the forebrain. Neuron 59(4):634-47. [PubMed: 18760699]  [MGI Ref ID J:149875]

Wang H; Wu LJ; Zhang F; Zhuo M. 2008. Roles of calcium-stimulated adenylyl cyclase and calmodulin-dependent protein kinase IV in the regulation of FMRP by group I metabotropic glutamate receptors. J Neurosci 28(17):4385-97. [PubMed: 18434517]  [MGI Ref ID J:134617]

Wang W; Zhu JZ; Chang KT; Min KT. 2012. DSCR1 interacts with FMRP and is required for spine morphogenesis and local protein synthesis. EMBO J 31(18):3655-66. [PubMed: 22863780]  [MGI Ref ID J:188613]

Wang X; Snape M; Klann E; Stone JG; Singh A; Petersen RB; Castellani RJ; Casadesus G; Smith MA; Zhu X. 2012. Activation of the extracellular signal-regulated kinase pathway contributes to the behavioral deficit of fragile x-syndrome. J Neurochem 121(4):672-9. [PubMed: 22393900]  [MGI Ref ID J:184475]

Weiler IJ; Spangler CC; Klintsova AY; Grossman AW; Kim SH; Bertaina-Anglade V; Khaliq H; de Vries FE; Lambers FA; Hatia F; Base CK; Greenough WT. 2004. Fragile X mental retardation protein is necessary for neurotransmitter-activated protein translation at synapses. Proc Natl Acad Sci U S A 101(50):17504-9. [PubMed: 15548614]  [MGI Ref ID J:94717]

Westmark CJ; Malter JS. 2007. FMRP Mediates mGluR5-Dependent Translation of Amyloid Precursor Protein. PLoS Biol 5(3):e52. [PubMed: 17298186]  [MGI Ref ID J:122019]

Westmark CJ; Westmark PR; O'Riordan KJ; Ray BC; Hervey CM; Salamat MS; Abozeid SH; Stein KM; Stodola LA; Tranfaglia M; Burger C; Berry-Kravis EM; Malter JS. 2011. Reversal of Fragile X Phenotypes by Manipulation of AbetaPP/Abeta Levels in Fmr1 Mice. PLoS One 6(10):e26549. [PubMed: 22046307]  [MGI Ref ID J:178076]

Wilson BM; Cox CL. 2007. Absence of metabotropic glutamate receptor-mediated plasticity in the neocortex of fragile X mice. Proc Natl Acad Sci U S A 104(7):2454-9. [PubMed: 17287348]  [MGI Ref ID J:119740]

Xu XL; Zong R; Li Z; Biswas MH; Fang Z; Nelson DL; Gao FB. 2011. FXR1P But Not FMRP Regulates the Levels of Mammalian Brain-Specific microRNA-9 and microRNA-124. J Neurosci 31(39):13705-9. [PubMed: 21957233]  [MGI Ref ID J:176126]

Yan QJ; Asafo-Adjei PK; Arnold HM; Brown RE; Bauchwitz RP. 2004. A phenotypic and molecular characterization of the fmr1-tm1Cgr fragile X mouse. Genes Brain Behav 3(6):337-59. [PubMed: 15544577]  [MGI Ref ID J:104517]

Yan QJ; Rammal M; Tranfaglia M; Bauchwitz RP. 2005. Suppression of two major Fragile X Syndrome mouse model phenotypes by the mGluR5 antagonist MPEP. Neuropharmacology 49(7):1053-66. [PubMed: 16054174]  [MGI Ref ID J:177984]

Yang Q; Feng B; Zhang K; Guo YY; Liu SB; Wu YM; Li XQ; Zhao MG. 2012. Excessive astrocyte-derived neurotrophin-3 contributes to the abnormal neuronal dendritic development in a mouse model of fragile X syndrome. PLoS Genet 8(12):e1003172. [PubMed: 23300470]  [MGI Ref ID J:194923]

Yun SW; Platholi J; Flaherty MS; Fu W; Kottmann AH; Toth M. 2006. Fmrp is required for the establishment of the startle response during the critical period of auditory development. Brain Res 1110(1):159-65. [PubMed: 16887106]  [MGI Ref ID J:113024]

Yuskaitis CJ; Beurel E; Jope RS. 2010. Evidence of reactive astrocytes but not peripheral immune system activation in a mouse model of Fragile X syndrome. Biochim Biophys Acta 1802(11):1006-12. [PubMed: 20600866]  [MGI Ref ID J:170023]

Yuskaitis CJ; Mines MA; King MK; Sweatt JD; Miller CA; Jope RS. 2010. Lithium ameliorates altered glycogen synthase kinase-3 and behavior in a mouse model of fragile X syndrome. Biochem Pharmacol 79(4):632-46. [PubMed: 19799873]  [MGI Ref ID J:158037]

Zalfa F; Eleuteri B; Dickson KS; Mercaldo V; De Rubeis S; di Penta A; Tabolacci E; Chiurazzi P; Neri G; Grant SG; Bagni C. 2007. A new function for the fragile X mental retardation protein in regulation of PSD-95 mRNA stability. Nat Neurosci 10(5):578-87. [PubMed: 17417632]  [MGI Ref ID J:121659]

Zang JB; Nosyreva ED; Spencer CM; Volk LJ; Musunuru K; Zhong R; Stone EF; Yuva-Paylor LA; Huber KM; Paylor R; Darnell JC; Darnell RB. 2009. A mouse model of the human Fragile X syndrome I304N mutation. PLoS Genet 5(12):e1000758. [PubMed: 20011099]  [MGI Ref ID J:155593]

Zang T; Maksimova MA; Cowan CW; Bassel-Duby R; Olson EN; Huber KM. 2013. Postsynaptic FMRP bidirectionally regulates excitatory synapses as a function of developmental age and MEF2 activity. Mol Cell Neurosci 56:39-49. [PubMed: 23511190]  [MGI Ref ID J:215299]

Zhang J; Fang Z; Jud C; Vansteensel MJ; Kaasik K; Lee CC; Albrecht U; Tamanini F; Meijer JH; Oostra BA; Nelson DL. 2008. Fragile X-related proteins regulate mammalian circadian behavioral rhythms. Am J Hum Genet 83(1):43-52. [PubMed: 18589395]  [MGI Ref ID J:139265]

Zhang J; Hou L; Klann E; Nelson DL. 2009. Altered hippocampal synaptic plasticity in the FMR1 gene family knockout mouse models. J Neurophysiol 101(5):2572-80. [PubMed: 19244359]  [MGI Ref ID J:164979]

Zhang L; Alger BE. 2010. Enhanced endocannabinoid signaling elevates neuronal excitability in fragile X syndrome. J Neurosci 30(16):5724-9. [PubMed: 20410124]  [MGI Ref ID J:159835]

Zhao MG; Toyoda H; Ko SW; Ding HK; Wu LJ; Zhuo M. 2005. Deficits in trace fear memory and long-term potentiation in a mouse model for fragile X syndrome. J Neurosci 25(32):7385-92. [PubMed: 16093389]  [MGI Ref ID J:100197]

Zhong J; Chuang SC; Bianchi R; Zhao W; Paul G; Thakkar P; Liu D; Fenton AA; Wong RK; Tiedge H. 2010. Regulatory BC1 RNA and the fragile X mental retardation protein: convergent functionality in brain. PLoS One 5(11):e15509. [PubMed: 21124905]  [MGI Ref ID J:167315]

de Vrij FM; Levenga J; van der Linde HC; Koekkoek SK; De Zeeuw CI; Nelson DL; Oostra BA; Willemsen R. 2008. Rescue of behavioral phenotype and neuronal protrusion morphology in Fmr1 KO mice. Neurobiol Dis 31(1):127-32. [PubMed: 18571098]  [MGI Ref ID J:139157]

el Bekay R; Romero-Zerbo Y; Decara J; Sanchez-Salido L; Del Arco-Herrera I; Rodriguez-de Fonseca F; de Diego-Otero Y. 2007. Enhanced markers of oxidative stress, altered antioxidants and NADPH-oxidase activation in brains from Fragile X mental retardation 1-deficient mice, a pathological model for Fragile X syndrome. Eur J Neurosci 26(11):3169-80. [PubMed: 18005058]  [MGI Ref ID J:130129]

Pde6b+ related

Dobkin C; Rabe A; Dumas R; El Idrissi A; Haubenstock H; Brown WT. 2000. Fmr1 knockout mouse has a distinctive strain-specific learning impairment. Neuroscience 100(2):423-9. [PubMed: 11008180]  [MGI Ref ID J:119166]

Ivanco TL; Greenough WT. 2002. Altered mossy fiber distributions in adult Fmr1 (FVB) knockout mice. Hippocampus 12(1):47-54. [PubMed: 11918288]  [MGI Ref ID J:113177]

Sakamoto K; McCluskey M; Wensel TG; Naggert JK; Nishina PM. 2009. New mouse models for recessive retinitis pigmentosa caused by mutations in the Pde6a gene. Hum Mol Genet 18(1):178-92. [PubMed: 18849587]  [MGI Ref ID J:142108]

Zhao MG; Toyoda H; Ko SW; Ding HK; Wu LJ; Zhuo M. 2005. Deficits in trace fear memory and long-term potentiation in a mouse model for fragile X syndrome. J Neurosci 25(32):7385-92. [PubMed: 16093389]  [MGI Ref ID J:100197]

Tyrc-ch related

Anderson PD; Lam MY; Poirier C; Bishop CE; Nadeau JH. 2009. The role of the mouse y chromosome on susceptibility to testicular germ cell tumors. Cancer Res 69(8):3614-8. [PubMed: 19351821]  [MGI Ref ID J:147731]

Beermann F; Ruppert S; Hummler E; Bosch FX; Muller G; Ruther U; Schutz G. 1990. Rescue of the albino phenotype by introduction of a functional tyrosinase gene into mice. EMBO J 9(9):2819-26. [PubMed: 2118105]  [MGI Ref ID J:19279]

Bhattacharya C; Aggarwal S; Zhu R; Kumar M; Zhao M; Meistrich ML; Matin A. 2007. The mouse dead-end gene isoform alpha is necessary for germ cell and embryonic viability. Biochem Biophys Res Commun 355(1):194-9. [PubMed: 17291453]  [MGI Ref ID J:118625]

Cattanach BM. 1961. A chemically-induced variegated-type position effect in the mouse. Z Vererbungsl 92:165-82. [PubMed: 13877379]  [MGI Ref ID J:160128]

Dobkin C; Rabe A; Dumas R; El Idrissi A; Haubenstock H; Brown WT. 2000. Fmr1 knockout mouse has a distinctive strain-specific learning impairment. Neuroscience 100(2):423-9. [PubMed: 11008180]  [MGI Ref ID J:119166]

Dunn LC. 1936. Studies on multiple allelomorphic series in the house mouse. I. Description of agouti and albino series of allelomorphs J Genet 33:443-53.  [MGI Ref ID J:22600]

Erickson RP; Gluecksohn-Waelsch S; Cori CF. 1968. Glucose-6-phosphatase deficiency caused by radiation-induced alleles at the albino locus in the mouse. Proc Natl Acad Sci U S A 59(2):437-44. [PubMed: 4296364]  [MGI Ref ID J:5063]

Errijgers V; Van Dam D; Gantois I; Van Ginneken CJ; Grossman AW; D'Hooge R; De Deyn PP; Kooy RF. 2007. FVB.129P2-Pde6b(+) Tyr(c-ch)/Ant, a sighted variant of the FVB/N mouse strain suitable for behavioral analysis. Genes Brain Behav 6(6):552-7. [PubMed: 17083330]  [MGI Ref ID J:137779]

Feldman HW. 1935. A fifth allelomorph in the albino series of the house mouse J Mammal 16:207-210.  [MGI Ref ID J:83666]

Feldman HW. 1922. A fourth allelomorph in the albino series in mice Am Naturalist 56:573-574.  [MGI Ref ID J:14850]

Klebig ML; Kwon BS; Rinchik EM. 1992. Physical analysis of murine albino deletions that disrupt liver-specific gene regulation or mesoderm development. Mamm Genome 2(1):51-63. [PubMed: 1543902]  [MGI Ref ID J:1540]

Laiosa MD; Lai ZW; Thurmond TS; Fiore NC; DeRossi C; Holdener BC; Gasiewicz TA; Silverstone AE. 2002. 2,3,7,8-tetrachlorodibenzo-p-dioxin causes alterations in lymphocyte development and thymic atrophy in hemopoietic chimeras generated from mice deficient in ARNT2. Toxicol Sci 69(1):117-24. [PubMed: 12215665]  [MGI Ref ID J:113951]

Lamoreux ML; Wakamatsu K; Ito S. 2001. Interaction of major coat color gene functions in mice as studied by chemical analysis of eumelanin and pheomelanin. Pigment Cell Res 14(1):23-31. [PubMed: 11277491]  [MGI Ref ID J:103803]

Lighthouse JK; Zhang L; Hsieh JC; Rosenquist T; Holdener BC. 2010. MESD is essential for apical localization of megalin/LRP2 in the visceral endoderm. Dev Dyn :. [PubMed: 21061374]  [MGI Ref ID J:168622]

Lossie AC; Nakamura H; Thomas SE; Justice MJ. 2005. Mutation of l7Rn3 shows that Odz4 is required for mouse gastrulation. Genetics 169(1):285-99. [PubMed: 15489520]  [MGI Ref ID J:96673]

Lyon MF. 1963. Attempts to test the inactive-X theory of dosage compensation in mammals Genet Res 4:93-103.  [MGI Ref ID J:272]

Medical Research Council (MRC) Harwell. 2012. Direct Data Submission 2012/01/26 MGI Direct Data Submission :.  [MGI Ref ID J:179802]

Moyer FH. 1966. Genetic variations in the fine structure and ontogeny of mouse melanin granules. Am Zool 6(1):43-66. [PubMed: 5902512]  [MGI Ref ID J:5001]

Pietropaolo S; Guilleminot A; Martin B; D'Amato FR; Crusio WE. 2011. Genetic-background modulation of core and variable autistic-like symptoms in Fmr1 knock-out mice. PLoS One 6(2):e17073. [PubMed: 21364941]  [MGI Ref ID J:171069]

RUSSELL ES. 1949. A quantitative histological study of the pigment found in the coat-color mutants of the house mouse; interdependence among the variable granule attributes. Genetics 34(2):133-45. [PubMed: 18117146]  [MGI Ref ID J:148461]

Russell ES. 1948. A Quantitative Histological Study of the Pigment Found in the Coat Color Mutants of the House Mouse. II. Estimates of the Total Volume of Pigment. Genetics 33(3):228-36. [PubMed: 17247280]  [MGI Ref ID J:148462]

Russell ES. 1946. A Quantitative Histological Study of the Pigment Found in the Coat-Color Mutants of the House Mouse. I. Variable Attributes of the Pigment Granules. Genetics 31(3):327-46. [PubMed: 17247200]  [MGI Ref ID J:148463]

Schedl A; Ruppert S; Kelsey G; Thies E; Niswander L; Magnuson T; Klebig ML; Rinchik EM; Schutz G. 1992. Chromosome jumping from flanking markers defines the minimal region for alf/hsdr-1 within the albino-deletion complex. Genomics 14(2):288-97. [PubMed: 1427845]  [MGI Ref ID J:2638]

Silvers WK. 1979. The Coat Colors of Mice; A Model for Mammalian Gene Action and Interaction. In: The Coat Colors of Mice. Springer-Verlag, New York.  [MGI Ref ID J:78801]

Staats J. 1985. Standardized Nomenclature for Inbred Strains of Mice: eighth listing. Cancer Res 45(3):945-77. [PubMed: 3971387]  [MGI Ref ID J:50296]

Strumbos JG; Brown MR; Kronengold J; Polley DB; Kaczmarek LK. 2010. Fragile X mental retardation protein is required for rapid experience-dependent regulation of the potassium channel Kv3.1b. J Neurosci 30(31):10263-71. [PubMed: 20685971]  [MGI Ref ID J:162850]

Sweet HO. 1987. Acromelanic (c<a>) Mouse News Lett 78:56.  [MGI Ref ID J:14994]

Takeuchi S; Yamamoto H; Takeuchi T. 1988. Expression of tyrosinase gene in mice Genome 30(Suppl 1):260 (Abstr.).  [MGI Ref ID J:30744]

Townsend D; Witkop CJ Jr; Mattson J. 1981. Tyrosinase subcellular distribution and kinetic parameters in wild type and C-locus mutant C57BL/6J mice. J Exp Zool 216(1):113-9. [PubMed: 6793688]  [MGI Ref ID J:6611]

Vasiliou V; Buetler T; Eaton DL; Nebert DW. 2000. Comparison of oxidative stress response parameters in newborn mouse liver versus simian virus 40 (SV40)-transformed hepatocyte cell lines. Biochem Pharmacol 59(6):703-12. [PubMed: 10677587]  [MGI Ref ID J:60274]

Vasiliou V; Reuter SF; Nebert DW. 1997. Extrahepatic expression of NAD(P)H:menadione oxidoreductase, UDP glucuronosyltransferase-1A6, microsomal aldehyde dehydrogenase, and hepatic nuclear factor-1 alpha mRNAs in ch/ch and 14CoS/14CoS mice. Biochem Biophys Res Commun 233(3):631-6. [PubMed: 9168903]  [MGI Ref ID J:40515]

Wu M; Rinchik EM; Wilkinson E; Johnson DK. 1997. Inherited somatic mosaicism caused by an intracisternal A particle insertion in the mouse tyrosinase gene. Proc Natl Acad Sci U S A 94(3):890-4. [PubMed: 9023352]  [MGI Ref ID J:38209]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX11

Colony Maintenance

Breeding & HusbandryThis strain is maintained by breeding homozygous females with hemizygous males. Fmr1 is an X linked gene. Expected coat color from breeding:albino
Mating SystemHomozygote x Hemizygote         (Female x Male)   01-MAR-06
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $199.90MaleHemizygous for Fmr1tm1Cgr  
$199.90FemaleHomozygous for Fmr1tm1Cgr  
Price per Pair (US dollars $)Pair Genotype
$399.80Homozygous for Fmr1tm1Cgr x Hemizygous for Fmr1tm1Cgr  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Cryopreserved

Frozen Products

Price (US dollars $)
Frozen Embryo $1650.00

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $259.90MaleHemizygous for Fmr1tm1Cgr  
$259.90FemaleHomozygous for Fmr1tm1Cgr  
Price per Pair (US dollars $)Pair Genotype
$519.80Homozygous for Fmr1tm1Cgr x Hemizygous for Fmr1tm1Cgr  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Cryopreserved

Frozen Products

Price (US dollars $)
Frozen Embryo $2145.00

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Control Information

  Control
   See control note: The control for this strain is STOCK# 4828, FVB.129P2-Pde6b+ Tyrc-ch/AntJ.
   004828 FVB.129P2-Pde6b+ Tyrc-ch/AntJ
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Important Note

This Fmr1 knockout is also available on a C57BL/6 genetic background: B6.129P2-Fmr1tm1Cgr/J (Stock No. 003025).

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


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The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
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JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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