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- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
- Terms of Use
Description
The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.
Strain Information
Type Congenic; Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Background Strain FVB/NJ Donor Strain B6,129P-Fmr1tm1Cgr (129P2 derived E14TG2a ES cell line) Generation N?+5F6p Donating Investigator IMR Colony, The Jackson Laboratory Appearance
albino
Related Genotype: Tyrc/TyrcImportant Note
This strain is homozygous for the retinal degeneration allele Pde6brd1.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.
Control Information
| Control | ||
|---|---|---|
| 001800 FVB/NJ | ||
| Considerations for Choosing Controls | ||
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)
004297 B6.CXB1-Pde6brd10/J 003647 B6EiC3Sn.BLiAF1 002802 C3.BLiA Pde6b+-Krd/J 001979 C3A.BLiA-Pde6b+.O20-Prph2Rd2/J 001912 C3A.BLiA-Pde6b+/J 003648 C3Sn.BLiA-Pde6b+/Dn 004766 C57BL/6J-Pde6brd1-2J/J 004828 FVB.129P2-Pde6b+ Tyrc-ch/AntJ 004808 STOCK Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J
Phenotype
Phenotype Information
Fragile X Mental Retardation Syndrome - Models with phenotypic similarity to human disease where etiologies involve orthologs.1
1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
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
- hyperactivity (MGI Ref ID J:19220)
- mutants show significantly more crossings through three infrared beams in an empty cage over 40 min
- increased exploration in new environment (MGI Ref ID J:19220)
- mutants exhibit more exploratory behavior than controls, displaying more line crossings in the lit compartment
Fmr1tm1Cgr/Fmr1tm1Cgr
involves: 129P2/OlaHsd
- behavior/neurological 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
- 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
- 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: 129P2/OlaHsd * 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)
This mouse can be used to support research in many areas including:Fmr1tm1Cgr related
Pde6brd1 relatedDevelopmental 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
Mouse/Human Gene Homologs
retinitis pigmentosa, autosomal recessive
Sensorineural Research
Retinal Degeneration
Genes & Alleles
Gene & Allele Information
| Allele Symbol | Fmr1tm1Cgr | ||
|---|---|---|---|
| Allele Name | targeted mutation 1, Ben Oostra | ||
| Allele Type | Targeted (knock-out) | ||
| Common Name(s) | FMRP KO; Fmr1 KO; FraX; fmr-tm1Cgr; | ||
| Strain of Origin | 129P2/OlaHsd | ||
| ES Cell Line Name | E14 | ||
| ES Cell Line Strain | 129P2/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 | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | Pdebrd1; rd; rd-1; rd1; rodless retina; | ||
Genotyping
Genotyping Information
Genotyping Protocols
Fmr1tm1Cgr, Separated PCR
Helpful Links
Genotyping resources and troubleshooting
References
References
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
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]
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]
Godfraind JM; Reyniers E; De Boulle K; D'Hooge R; De Deyn PP; Bakker CE; Oostra BA; Kooy RF; Willems PJ. 1996. Long-term potentiation in the hippocampus of fragile X knockout mice. Am J Med Genet 64(2):246-51. [PubMed: 8844057] [MGI Ref ID J:34552]
Kooy RF; D'Hooge R; Reyniers E; Bakker CE; Nagels G; De Boulle K; Storm K; Clincke G; De Deyn PP; Oostra BA; Willems PJ. 1996. Transgenic mouse model for the fragile X syndrome. Am J Med Genet 64(2):241-5. [PubMed: 8844056] [MGI Ref ID J:34449]
Oostra BA; Willems PJ. 1995. A fragile gene. Bioessays 17(11):941-7. [PubMed: 8526888] [MGI Ref ID J:41538]
Paradee W; Melikian HE; Rasmussen DL; Kenneson A; Conn PJ; Warren ST. 1999. Fragile X mouse: strain effects of knockout phenotype and evidence suggesting deficient amygdala function. Neuroscience 94(1):185-92. [PubMed: 10613508] [MGI Ref ID J:59781]
Qin M; Kang J; Smith CB. 2002. Increased rates of cerebral glucose metabolism in a mouse model of fragile X mental retardation. Proc Natl Acad Sci U S A 99(24):15758-63. [PubMed: 12427968] [MGI Ref ID J:80518]
Reyniers E; Van Bockstaele DR; De Boulle K; Kooy RF; Bakker CE; Oostra BA; Willems PJ. 1996. Mean corpuscular hemoglobin is not increased in Fmr1 knockout mice. Hum Genet 97(1):49-50. [PubMed: 8557260] [MGI Ref ID J:31093]
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]
Fmr1tm1Cgr relatedAntar 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]
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]
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]
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]
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]
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]
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]
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]
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]
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]
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]
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]
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]
Gantois I; Vandesompele J; Speleman F; Reyniers E; D'Hooge R; Severijnen LA; Willemsen R; Tassone F; Kooy RF. 2006. Expression profiling suggests underexpression of the GABA(A) receptor subunit delta in the fragile X knockout mouse model Neurobiol Dis 21:346-357. [PubMed: 16199166] [MGI Ref ID J:105740]
Giuffrida R; Musumeci S; D'Antoni S; Bonaccorso CM; Giuffrida-Stella AM; Oostra BA; Catania MV. 2005. A reduced number of metabotropic glutamate subtype 5 receptors are associated with constitutive homer proteins in a mouse model of fragile X syndrome. J Neurosci 25(39):8908-16. [PubMed: 16192381] [MGI Ref ID J:101346]
Godfraind JM; Reyniers E; De Boulle K; D'Hooge R; De Deyn PP; Bakker CE; Oostra BA; Kooy RF; Willems PJ. 1996. Long-term potentiation in the hippocampus of fragile X knockout mice. Am J Med Genet 64(2):246-51. [PubMed: 8844057] [MGI Ref ID J:34552]
Grossman AW; Elisseou NM; McKinney BC; Greenough WT. 2006. Hippocampal pyramidal cells in adult Fmr1 knockout mice exhibit an immature-appearing profile of dendritic spines. Brain Res 1084(1):158-164. [PubMed: 16574084] [MGI Ref ID J:108977]
Gruss M; Braun K. 2004. Age- and region-specific imbalances of basal amino acids and monoamine metabolism in limbic regions of female Fmr1 knock-out mice. Neurochem Int 45(1):81-8. [PubMed: 15082225] [MGI Ref ID J:101787]
Hanson JE; Madison DV. 2007. Presynaptic FMR1 genotype influences the degree of synaptic connectivity in a mosaic mouse model of fragile X syndrome. J Neurosci 27(15):4014-8. [PubMed: 17428978] [MGI Ref ID J:143837]
Hayashi ML; Rao BS; Seo JS; Choi HS; Dolan BM; Choi SY; Chattarji S; Tonegawa S. 2007. Inhibition of p21-activated kinase rescues symptoms of fragile X syndrome in mice. Proc Natl Acad Sci U S A 104(27):11489-11494. [PubMed: 17592139] [MGI Ref ID J:122818]
Hou L; Antion MD; Hu D; Spencer CM; Paylor R; Klann E. 2006. Dynamic translational and proteasomal regulation of fragile X mental retardation protein controls mGluR-dependent long-term depression. Neuron 51(4):441-54. [PubMed: 16908410] [MGI Ref ID J:122974]
Hu H; Qin Y; Bochorishvili G; Zhu Y; van Aelst L; Zhu JJ. 2008. Ras signaling mechanisms underlying impaired GluR1-dependent plasticity associated with fragile X syndrome. J Neurosci 28(31):7847-62. [PubMed: 18667617] [MGI Ref ID J:139516]
Huber KM; Gallagher SM; Warren ST; Bear MF. 2002. Altered synaptic plasticity in a mouse model of fragile X mental retardation. Proc Natl Acad Sci U S A 99(11):7746-50. [PubMed: 12032354] [MGI Ref ID J:76865]
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]
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]
Kim SH; Markham JA; Weiler IJ; Greenough WT. 2008. Aberrant early-phase ERK inactivation impedes neuronal function in fragile X syndrome. Proc Natl Acad Sci U S A 105(11):4429-34. [PubMed: 18332424] [MGI Ref ID J:133336]
Koekkoek SK; Yamaguchi K; Milojkovic BA; Dortland BR; Ruigrok TJ; Maex R; De Graaf W; Smit AE; VanderWerf F; Bakker CE; Willemsen R; Ikeda T; Kakizawa S; Onodera K; Nelson DL; Mientjes E; Joosten M; De Schutter E; Oostra BA; Ito M; De Zeeuw CI. 2005. Deletion of FMR1 in Purkinje cells enhances parallel fiber LTD, enlarges spines, and attenuates cerebellar eyelid conditioning in Fragile X syndrome. Neuron 47(3):339-52. [PubMed: 16055059] [MGI Ref ID J:101021]
Kooy RF; D'Hooge R; Reyniers E; Bakker CE; Nagels G; De Boulle K; Storm K; Clincke G; De Deyn PP; Oostra BA; Willems PJ. 1996. Transgenic mouse model for the fragile X syndrome. Am J Med Genet 64(2):241-5. [PubMed: 8844056] [MGI Ref ID J:34449]
Kooy RF; Reyniers E; Verhoye M; Sijbers J; Bakker CE; Oostra BA; Willems PJ; Van Der Linden A. 1999. Neuroanatomy of the fragile X knockout mouse brain studied using in vivo high resolution magnetic resonance imaging. Eur J Hum Genet 7(5):526-32. [PubMed: 10439957] [MGI Ref ID J:103315]
Larson J; Jessen RE; Kim D; Fine AK; du Hoffmann J. 2005. Age-dependent and selective impairment of long-term potentiation in the anterior piriform cortex of mice lacking the fragile X mental retardation protein. J Neurosci 25(41):9460-9. [PubMed: 16221856] [MGI Ref ID J:101394]
Larson J; Kim D; Patel RC; Floreani C. 2008. Olfactory discrimination learning in mice lacking the fragile X mental retardation protein. Neurobiol Learn Mem 90(1):90-102. [PubMed: 18289890] [MGI Ref ID J:139604]
Lauterborn JC. 2004. Stress induced changes in cortical and hypothalamic c-fos expression are altered in fragile X mutant mice. Brain Res Mol Brain Res 131(1-2):101-9. [PubMed: 15530658] [MGI Ref ID J:109325]
Lauterborn JC; Rex CS; Kramar E; Chen LY; Pandyarajan V; Lynch G; Gall CM. 2007. Brain-derived neurotrophic factor rescues synaptic plasticity in a mouse model of fragile X syndrome. J Neurosci 27(40):10685-94. [PubMed: 17913902] [MGI Ref ID J:125595]
Li J; Pelletier MR; Perez Velazquez JL; Carlen PL. 2002. Reduced Cortical Synaptic Plasticity and GluR1 Expression Associated with Fragile X Mental Retardation Protein Deficiency. Mol Cell Neurosci 19(2):138-51. [PubMed: 11860268] [MGI Ref ID J:75442]
Liao L; Park SK; Xu T; Vanderklish P; Yates JR rd. 2008. Quantitative proteomic analysis of primary neurons reveals diverse changes in synaptic protein content in fmr1 knockout mice. Proc Natl Acad Sci U S A 105(40):15281-6. [PubMed: 18829439] [MGI Ref ID J:141828]
Lu R; Wang H; Liang Z; Ku L; O'donnell WT; Li W; Warren ST; Feng Y. 2004. The fragile X protein controls microtubule-associated protein 1B translation and microtubule stability in brain neuron development. Proc Natl Acad Sci U S A 101(42):15201-6. [PubMed: 15475576] [MGI Ref ID J:93541]
Markham JA; Beckel-Mitchener AC; Estrada CM; Greenough WT. 2006. Corticosterone response to acute stress in a mouse model of Fragile X syndrome. Psychoneuroendocrinology 31(6):781-5. [PubMed: 16621323] [MGI Ref ID J:112825]
Meredith RM; Holmgren CD; Weidum M; Burnashev N; Mansvelder HD. 2007. Increased threshold for spike-timing-dependent plasticity is caused by unreliable calcium signaling in mice lacking fragile X gene FMR1. Neuron 54(4):627-38. [PubMed: 17521574] [MGI Ref ID J:122303]
Mineur YS; Huynh LX; Crusio WE. 2006. Social behavior deficits in the Fmr1 mutant mouse. Behav Brain Res 168(1):172-5. [PubMed: 16343653] [MGI Ref ID J:105026]
Mineur YS; Sluyter F; de Wit S; Oostra BA; Crusio WE. 2002. Behavioral and neuroanatomical characterization of the Fmr1 knockout mouse. Hippocampus 12(1):39-46. [PubMed: 11918286] [MGI Ref ID J:113306]
Miyashiro KY; Beckel-Mitchener A; Purk TP; Becker KG; Barret T; Liu L; Carbonetto S; Weiler IJ; Greenough WT; Eberwine J. 2003. RNA cargoes associating with FMRP reveal deficits in cellular functioning in Fmr1 null mice. Neuron 37(3):417-31. [PubMed: 12575950] [MGI Ref ID J:107689]
Moy SS; Nadler JJ; Young NB; Nonneman RJ; Grossman AW; Murphy DL; D'Ercole AJ; Crawley JN; Magnuson TR; Lauder JM. 2009. Social approach in genetically engineered mouse lines relevant to autism. Genes Brain Behav 8(2):129-42. [PubMed: 19016890] [MGI Ref ID J:151144]
Muddashetty RS; Kelic S; Gross C; Xu M; Bassell GJ. 2007. Dysregulated metabotropic glutamate receptor-dependent translation of AMPA receptor and postsynaptic density-95 mRNAs at synapses in a mouse model of fragile X syndrome. J Neurosci 27(20):5338-48. [PubMed: 17507556] [MGI Ref ID J:143833]
Musumeci SA; Bosco P; Calabrese G; Bakker C; De Sarro GB; Elia M; Ferri R; Oostra BA. 2000. Audiogenic seizures susceptibility in transgenic mice with fragile X syndrome. Epilepsia 41(1):19-23. [PubMed: 10643918] [MGI Ref ID J:60043]
Nielsen DM; Derber WJ; McClellan DA; Crnic LS. 2002. Alterations in the auditory startle response in Fmr1 targeted mutant mouse models of fragile X syndrome. Brain Res 927(1):8-17. [PubMed: 11814427] [MGI Ref ID J:74592]
Nimchinsky EA; Oberlander AM; Svoboda K. 2001. Abnormal development of dendritic spines in FMR1 knock-out mice. J Neurosci 21(14):5139-46. [PubMed: 11438589] [MGI Ref ID J:70178]
Nosyreva ED; Huber KM. 2006. Metabotropic receptor-dependent long-term depression persists in the absence of protein synthesis in the mouse model of fragile X syndrome. J Neurophysiol 95(5):3291-5. [PubMed: 16452252] [MGI Ref ID J:135796]
Pacey LK; Doering LC. 2007. Developmental expression of FMRP in the astrocyte lineage: implications for fragile X syndrome. Glia 55(15):1601-9. [PubMed: 17823967] [MGI Ref ID J:127940]
Paradee W; Melikian HE; Rasmussen DL; Kenneson A; Conn PJ; Warren ST. 1999. Fragile X mouse: strain effects of knockout phenotype and evidence suggesting deficient amygdala function. Neuroscience 94(1):185-92. [PubMed: 10613508] [MGI Ref ID J:59781]
Park S; Park JM; Kim S; Kim JA; Shepherd JD; Smith-Hicks CL; Chowdhury S; Kaufmann W; Kuhl D; Ryazanov AG; Huganir RL; Linden DJ; Worley PF. 2008. Elongation factor 2 and fragile X mental retardation protein control the dynamic translation of Arc/Arg3.1 essential for mGluR-LTD. Neuron 59(1):70-83. [PubMed: 18614030] [MGI Ref ID J:145490]
Pfeiffer BE; Huber KM. 2007. Fragile X mental retardation protein induces synapse loss through acute postsynaptic translational regulation. J Neurosci 27(12):3120-30. [PubMed: 17376973] [MGI Ref ID J:143842]
Price TJ; Rashid MH; Millecamps M; Sanoja R; Entrena JM; Cervero F. 2007. Decreased nociceptive sensitization in mice lacking the fragile X mental retardation protein: role of mGluR1/5 and mTOR. J Neurosci 27(51):13958-67. [PubMed: 18094233] [MGI Ref ID J:129270]
Qin M; Kang J; Burlin TV; Jiang C; Smith CB. 2005. Postadolescent changes in regional cerebral protein synthesis: an in vivo study in the FMR1 null mouse. J Neurosci 25(20):5087-95. [PubMed: 15901791] [MGI Ref ID J:98726]
Qin M; Kang J; Smith CB. 2005. A null mutation for Fmr1 in female mice: effects on regional cerebral metabolic rate for glucose and relationship to behavior. Neuroscience 135(3):999-1009. [PubMed: 16154294] [MGI Ref ID J:104426]
Qin M; Kang J; Smith CB. 2002. Increased rates of cerebral glucose metabolism in a mouse model of fragile X mental retardation. Proc Natl Acad Sci U S A 99(24):15758-63. [PubMed: 12427968] [MGI Ref ID J:80518]
Restivo L; Ferrari F; Passino E; Sgobio C; Bock J; Oostra BA; Bagni C; Ammassari-Teule M. 2005. Enriched environment promotes behavioral and morphological recovery in a mouse model for the fragile X syndrome. Proc Natl Acad Sci U S A 102(32):11557-62. [PubMed: 16076950] [MGI Ref ID J:100841]
Reyniers E; Van Bockstaele DR; De Boulle K; Kooy RF; Bakker CE; Oostra BA; Willems PJ. 1996. Mean corpuscular hemoglobin is not increased in Fmr1 knockout mice. Hum Genet 97(1):49-50. [PubMed: 8557260] [MGI Ref ID J:31093]
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]
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]
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]
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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]
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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]
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Pricing
| Pricing for USA, Canada and Mexico shipping destinations |
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Animals Provided
Price (US dollars $) Cryorecovery Fee $1900.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
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Animals Provided
Price (US dollars $) Cryorecovery Fee $2470.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Additional Supply Details
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Supply Details
| Standard Supply | Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information. |
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| Supply Notes |
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| Important Note | |
| This strain is homozygous for the retinal degeneration allele Pde6brd1. | |
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| Control | ||
|---|---|---|
| 001800 FVB/NJ | ||
| Considerations for Choosing Controls | ||
| USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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