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Strain Name:

FVB.129P2(B6)-Fmr1tm1Cgr/J

Stock Number:

003024

Availability:

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General Terms and Conditions

Genes & Alleles   Fmr1;   Fmr1tm1Cgr;   Pde6b;   Pde6brd1;

Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Type JAX® GEMM® Strain - Targeted Mutation
Specieslaboratory mouse
Background Strain FVB/NJ
Donor Strain B6,129P-Fmr1tm1Cgr (129P2 derived E14TG2a ES cell line)
Donating Investigator IMR Colony,   The Jackson Laboratory
GenerationN?+5F6p

Appearance
albino
Related Genotype: Tyrc/Tyrc

Important Note
This strain is homozygous for the retinal degeneration allele Pde6brd1.

Strain Description
Mice homozygous for the Fmr1tm1Cg targeted mutation show macroorchidism (enlarged testes), learning deficits, and hyperactivity. Macroorchidism in caused by an increased rate of Sertoli cell proliferation during embryogenesis which may be independent of FSH signalling. Comparison of homozygotes to wildtype littermates in hidden- and visible-platform water maze learning showed deficits in spatial learning and motor performance.

Related Disease (OMIM) Terms

Fragile X Mental Retardation Syndrome
Mammalian Phenotype Terms assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Fmr1tm1Cgr/Fmr1tm1Cgr

        involves: 129P2/OlaHsd * C57BL/6J
  • behavior/neurological phenotype
  • abnormal learning/ memory (MGI Ref ID J:19220)
  • hyperactivity (MGI Ref ID J:19220)
  • endocrine/exocrine gland phenotype
  • enlarged testes (MGI Ref ID J:19220)
    • enlarged testes; however, both males and females were fertile
  • reproductive system phenotype
  • enlarged testes (MGI Ref ID J:19220)
    • enlarged testes; however, both males and females were fertile

Fmr1tm1Cgr/Fmr1tm1Cgr

        involves: 129P2/OlaHsd
  • behavior/neurological phenotype
  • abnormal eye blink conditioning behavior (MGI Ref ID J:101021)
    • 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
  • abnormal learning/ memory (MGI Ref ID J:34449)
  • increased startle reflex (MGI Ref ID J:101021)
    • in all eye blink conditioning training session the percentage and peak amplitudes of the startle responses were higher
  • nervous system phenotype
  • abnormal Purkinje cell morphology (MGI Ref ID J:101021)
    • the percentage of single climbing fiber innervation is increased, the length of spine heads and necks is increased, and spines are more irregular
  • abnormal long term depression (MGI Ref ID J:101021)
    • induction of long term depression in Purkinje cells is significantly enhanced when stimulating parallel fibers
  • endocrine/exocrine gland phenotype
  • enlarged testes (MGI Ref ID J:34449)
    • progressive enlargement resulting in testes that were 34% bigger than those of wild type by 168 to 170 days of age
    • however, both males and females were fertile
  • reproductive system phenotype
  • enlarged testes (MGI Ref ID J:34449)
    • progressive enlargement resulting in testes that were 34% bigger than those of wild type by 168 to 170 days of age
    • however, both males and females were fertile
  • hearing/vestibular/ear phenotype
  • increased startle reflex (MGI Ref ID J:101021)
    • in all eye blink conditioning training session the percentage and peak amplitudes of the startle responses were higher

Fmr1tm1Cgr/Y

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

Gene & Allele Details

Allele Symbol Fmr1tm1Cgr
Allele Name targeted mutation 1, Ben Oostra
Common Name(s) FMRP KO; Fmr1 KO; FraX; fmr-tm1Cgr;
Strain of Origin129P2/OlaHsd
ES Cell Line NameE14
ES Cell Line Strain129P2/OlaHsd
Gene Symbol and Name Fmr1, fragile X mental retardation syndrome 1 homolog
Chromosome X
Gene Common Name(s) FMRP; FRAXA; Fmr-1; MGC87458; 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 Pde6brd1
Allele Name retinal degeneration 1
Common Name(s) rd; rd-1; rd1; rodless retina;

Control Information

  Allele   Control
 Fmr1tm1Cgr  001800 FVB/NJ
 
  Considerations for Choosing Controls

Genotyping Protocols

Fmr1tm1Cgr

Colony Maintenance

Diet Information LabDiet® 5K52/5K67

Related Strains

Strains carrying   Fmr1tm1Cgr allele
003025   B6.129P2-Fmr1tm1Cgr/J
004624   FVB.129P2-Fmr1tm1Cgr/J
002700   FVB;129P-Fmr1tm1Cgr/J
View Strains carrying   Fmr1tm1Cgr     (3 strains)

Strains carrying   Pde6brd1 allele
004202   B6.C3 Pde6brd1 Hps4le/+ +-Lmx1adr-8J/J
000002   B6.C3-Pde6brd1 Hps4le/J
001022   B6C3FeF1/J a/a
000652   BDP/J
000653   BUB/BnJ
002439   C3.129P2(B6)-B2mtm1Unc/J
005494   C3.129S1(B6)-Grm1rcw/J
000480   C3.MRL-Faslpr/J
001957   C3A Pde6brd1.O20/A-Prph2Rd2/J
005973   C3Bir.129P2(B6)-Il10C3Bir/LtJ
004326   C3Bir.129P2(B6)-Il10tm1Cgn/Lt
003968   C3Bir.129P2(B6)-Il10tm1Cgn/LtJ
001906   C3Ga.Cg-Catb/J
001904   C3H-Atcayji-hes/J
000659   C3H/HeJ
000784   C3H/HeJ-Faslgld/J
000509   C3H/HeJ-Lystbg-2J/J
002433   C3H/HeJ-Spnb4qv-lnd2J/J
005972   C3H/HeJBirLtJ
001824   C3H/HeJSxJ
000635   C3H/HeOuJ
000474   C3H/HeSn
001431   C3H/HeSn-ocd/J
000661   C3H/HeSnJ
002235   C3H/HeSnJ-Ctnna2cdf/J
002333   C3H/HeSnJ-gri/J
006435   C3HeB.SW-Soaa/MonJ
000658   C3HeB/FeJ
001576   C3HeB/FeJ-Atp7btx-J/J
002588   C3HeB/FeJ-Eya1bor/J
001533   C3HeB/FeJ-Mc1rE-so Gli3Xt-J/J
001886   C3HeB/FeJLe a/a-gnd/J
001908   C3HfB/BiJ
001502   C3Sn.B6-Epha4rb/J
001547   C3Sn.Cg-Cm/J
000656   CBA/J
000813   CBA/J-Atp7aMo-pew/J
000660   DA/HuSnJ
000023   FL/1ReJ
000025   FL/4ReJ
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
003257   FVB/N-Tg(GFAPGFP)14Mes/J
002374   FVB/N-Tg(MMTV-PyVT)634Mul/J
002856   FVB/N-Tg(TIE2-lacZ)182Sato/J
002384   FVB/N-Tg(UcpDta)1Kz/J
001800   FVB/NJ
003487   FVB/NJ-Tg(XGFAP-lacZ)3Mes/J
001491   FVB/NMob
000734   MOLD/RkJ
000550   MOLF/EiJ
002423   NON/ShiLtJ
000679   P/J
000680   PL/J
100299   PLSJLF1/J
000269   SB/LeJ
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
100401   WCB6F1/J KitlSl KitlSl-d
View Strains carrying   Pde6brd1     (74 strains)

View Strains carrying other alleles of Pde6b     (8 strains)

Additional Web Information

Congenic Nomenclature

Research Applications

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

Fmr1tm1Cgr related

Developmental Biology Research
Internal/Organ Defects (multiple)

Mouse/Human Gene Homologs
fragile site mental retardation 1

Neurobiology Research
Behavioral and Learning Defects
Fragile X Mental Retardation Syndrome

Pde6brd1 related

Mouse/Human Gene Homologs
retinitis pigmentosa, autosomal recessive

Sensorineural Research
Retinal Degeneration

References

Selected Reference(s)

The Dutch-Belgium Fragile X Consortium: 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. 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

Price and Supply Information

Strain Name: FVB.129P2(B6)-Fmr1tm1Cgr/J
Stock Number: 003024

Price Details

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Supply Details

Standard SupplyRepository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.
Supply Notes Cryorecovery - Standard.
The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery.

Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services: Tel: 1-800-422-6423 or 1-207-288-5845; Email: jaxservices@jax.org.
This strain is included in the Induced Mutant Resource Colony collection.
Genomic DNA is available for this strain from the Mouse DNA Resource.

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Control InformationView Control Information in Strain Details.

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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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