Strain Name:

B6.BR-Agtpbp1pcd/J

Stock Number:

000537

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Mice homozygous for the Agtpbp1pcd spontaneous mutation show a moderate ataxia with degeneration of Purkinje cells.

Description

Strain Information

Former Names B6.BR-Nna1pcd    (Changed: 15-DEC-04 )
Type Congenic; Mutant Strain; Spontaneous Mutation;
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Mating SystemOvarian Transplant-Cross-Intercross         (Female x Male)   01-MAR-06
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain C57BR/cdJ
GenerationN58F1 (25-OCT-13)
Generation Definitions

Appearance
black, ataxic
Related Genotype: a/a Agtpbp1pcd/Agtpbp1pcd

black, unaffected
Related Genotype: a/a Agtpbp1pcd/+ or a/a ?/+

Description
Mice homozygous for the Purkinje cell degeneration spontaneous mutation (Agtpbp1pcd) show a moderate ataxia beginning at 3 to 4 weeks. Homozygous mutant mice are somewhat smaller than normal but may live a fairly normal life span. Males have abnormal sperm and are sterile. Females are fertile but are poor breeders. There is rapid degeneration of nearly all Purkinje cells beginning at 15 to 18 days, and a slower degeneration of the photoreceptor cells of the retina and mitral cells of the olfactory bulb. Degeneration of Purkinje cells is followed by partial loss of granule cells. Discrete serotonin-immunoreactive fibers, which ascend to all three layers of the cerebellar cortex in normal controls, are of much higher density and form multidirectional contours in homozygotes. In the retina of homozygous mutant mice, pycnotic nuclei begin to appear in the photoreceptor cells between 18 and 25 days, and the outer rod segments become disorganized. Degeneration of the photoreceptor cells proceeds slowly to completeness over the course of a year.

Development
The Agtpbp1pcd mutation, originally designated pcd, occurred in the C57BR/cdJ strain at The Jackson Laboratory. Homozygous females were initially mated to C3H/ HeJ males and the F1-progeny were intercrossed to produce homozygous F2 females. These females were then crossed back to C57BR/cdJ males and the Agtpbp1pcd mutation was maintained thereafter by brother-sister matings until backcrossed to the C57BL/6J strain.

Control Information

  Control
   Untyped from the colony
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Agtpbp1pcd allele
001037   B6C3Fe a/a-Agtpbp1pcd/J
View Strains carrying   Agtpbp1pcd     (1 strain)

Strains carrying other alleles of Agtpbp1
005348   BALB/cByJ Agtpbp1pcd-3J-Bmp5cfe-se6J/GrsrJ
003237   BALB/cByJ-Agtpbp1pcd-3J/J
005011   C57BL/6J-Agtpbp1pcd-6J/J
004518   DBA/2J-Agtpbp1pcd-5J/GrsrJ
View Strains carrying other alleles of Agtpbp1     (4 strains)

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

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

Agtpbp1pcd/Agtpbp1pcd

        involves: C57BR/cdJ * CBA
  • behavior/neurological phenotype
  • ataxia
    • age of onset, 3 - 4 weeks   (MGI Ref ID J:5613)
  • nervous system phenotype
  • abnormal cerebellar Purkinje cell layer
    • symmetrical regions of more resistant cells but most disappear eventually   (MGI Ref ID J:106414)
    • climbing fibers never contact Purkinje cells   (MGI Ref ID J:23733)
    • Purkinje cell degeneration
      • age of onset, 15 - 18 days   (MGI Ref ID J:5613)
    • abnormal Purkinje cell dendrite morphology
      • atrophic dendritic trees   (MGI Ref ID J:23733)
    • decreased Purkinje cell number
      • cell loss begins at 3 weeks of age and progresses rapidly   (MGI Ref ID J:106414)
      • about 1% of initial population remains at 2 months of age   (MGI Ref ID J:106414)
  • abnormal cerebellar granule layer morphology
    • granule cell degeneration, partial loss following Purkinje cell degeneration   (MGI Ref ID J:5613)
  • abnormal olfactory bulb morphology
    • olfactory mitral cell degeneration, slow and progressive   (MGI Ref ID J:5613)
  • abnormal thalamus morphology
    • thalamic neuronal degeneration, age of onset 50 - 60 days   (MGI Ref ID J:5613)
  • retinal photoreceptor degeneration
    • photoreceptor cell degeneration, age of onset, 18 - 25 days   (MGI Ref ID J:5613)
    • complete over the course of 1 year   (MGI Ref ID J:5613)
  • vision/eye phenotype
  • retinal photoreceptor degeneration
    • photoreceptor cell degeneration, age of onset, 18 - 25 days   (MGI Ref ID J:5613)
    • complete over the course of 1 year   (MGI Ref ID J:5613)
  • reproductive system phenotype
  • abnormal male germ cell morphology
    • of the few sperm found, these were degenerated   (MGI Ref ID J:5613)
    • oligozoospermia   (MGI Ref ID J:5613)
  • asthenozoospermia   (MGI Ref ID J:5613)
  • male infertility   (MGI Ref ID J:5613)
  • reduced female fertility
    • females were poor breeders   (MGI Ref ID J:5613)

Agtpbp1pcd/Agtpbp1pcd

        involves: C57BR/cdJ
  • nervous system phenotype
  • abnormal cochlear VIII nucleus morphology
    • few cartwheel cells in the dorsal cochlear nucleus   (MGI Ref ID J:121314)
  • decreased Purkinje cell number
    • few Purkinje cell   (MGI Ref ID J:121314)
View Research Applications

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

Agtpbp1pcd related

Neurobiology Research
Ataxia (Movement) Defects
Cerebellar Defects
      Purkinje cell defect
Neurodegeneration

Sensorineural Research
Retinal Degeneration

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Agtpbp1pcd
Allele Name Purkinje cell degeneration
Allele Type Spontaneous (Not Specified)
Common Name(s) Nna1pcd; pcd; pcd1J;
Strain of OriginC57BR/cdJ
Gene Symbol and Name Agtpbp1, ATP/GTP binding protein 1
Chromosome 13
Gene Common Name(s) 1700020N17Rik; 2310001G17Rik; 2900054O13Rik; 4930445M19Rik; 5730402G09Rik; BB114605; CCP1; NNA1; Purkinje cell degeneration; RIKEN cDNA 1700020N17 gene; RIKEN cDNA 2310001G17 gene; RIKEN cDNA 2900054O13 gene; RIKEN cDNA 4930445M19 gene; RIKEN cDNA 5730402G09 gene; expressed sequence BB114605; nmf243; pcd;
Molecular Note No overt mutations were noted in the coding region for this allele. Northern analysis failed to detect transcript in all tissues except for testis, where reduced levels were noted. Authors note that the mutation is likely in a regulatory region of the gene. [MGI Ref ID J:74929]

Genotyping

Genotyping Information


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References

References provided by MGI

Additional References

Chang B; Hawes NL; Hurd RE; Davisson MT; Nusinowitz S; Heckenlively JR. 2002. Retinal degeneration mutants in the mouse. Vision Res 42(4):517-25. [PubMed: 11853768]  [MGI Ref ID J:75095]

Fernandez-Gonzalez A; La Spada AR; Treadaway J; Higdon JC; Harris BS; Sidman RL; Morgan JI; Zuo J. 2002. Purkinje cell degeneration (pcd) phenotypes caused by mutations in the axotomy-induced gene, Nna1. Science 295(5561):1904-6. [PubMed: 11884758]  [MGI Ref ID J:74929]

Harris A; Morgan JI; Pecot M; Soumare A; Osborne A; Soares HD. 2000. Regenerating motor neurons express Nna1, a novel ATP/GTP-binding protein related to zinc carboxypeptidases. Mol Cell Neurosci 16(5):578-96. [PubMed: 11083920]  [MGI Ref ID J:74936]

Mullen RJ; Eicher EM; Sidman RL. 1976. Purkinje cell degeneration, a new neurological mutation in the mouse. Proc Natl Acad Sci U S A 73(1):208-12. [PubMed: 1061118]  [MGI Ref ID J:5613]

O'Gorman S. 1985. Degeneration of thalamic neurons in Purkinje cell degeneration mutant mice. II. Cytology of neuron loss. J Comp Neurol 234(3):298-316. [PubMed: 3988986]  [MGI Ref ID J:7820]

Agtpbp1pcd related

Bakalian A; Kopmels B; Messer A; Fradelizi D; Delhaye-Bouchaud N; Wollman E; Mariani J. 1992. Peripheral macrophage abnormalities in mutant mice with spinocerebellar degeneration. Res Immunol 143(1):129-39. [PubMed: 1565842]  [MGI Ref ID J:2228]

Baltanas FC; Berciano MT; Valero J; Gomez C; Diaz D; Alonso JR; Lafarga M; Weruaga E. 2013. Differential glial activation during the degeneration of Purkinje cells and mitral cells in the PCD mutant mice. Glia 61(2):254-72. [PubMed: 23047288]  [MGI Ref ID J:191138]

Baltanas FC; Casafont I; Lafarga V; Weruaga E; Alonso JR; Berciano MT; Lafarga M. 2011. Purkinje cell degeneration in pcd mice reveals large scale chromatin reorganization and gene silencing linked to defective DNA repair. J Biol Chem 286(32):28287-302. [PubMed: 21700704]  [MGI Ref ID J:175919]

Bartolomei JC; Greer CA. 1998. The organization of piriform cortex and the lateral olfactory tract following the loss of mitral cells in PCD mice. Exp Neurol 154(2):537-50. [PubMed: 9878189]  [MGI Ref ID J:52050]

Baurle J; Grover BG; Grusser-Cornehls U. 1992. Plasticity of GABAergic terminals in Deiters' nucleus of weaver mutant and normal mice: a quantitative light microscopic study. Brain Res 591(2):305-18. [PubMed: 1446244]  [MGI Ref ID J:2651]

Baurle J; Grusser-Cornehls U. 1994. Calbindin D-28k in the lateral vestibular nucleus of mutant mice as a tool to reveal Purkinje cell plasticity. Neurosci Lett 167(1-2):85-8. [PubMed: 8177535]  [MGI Ref ID J:19042]

Baurle J; Grusser-Cornehls U. 1997. Differential number of glycine- and GABA-immunopositive neurons and terminals in the deep cerebellar nuclei of normal and Purkinje cell degeneration mutant mice. J Comp Neurol 382(4):443-58. [PubMed: 9184992]  [MGI Ref ID J:41323]

Baurle J; Guldin W. 1998. Vestibular ganglion neurons survive the loss of their cerebellar targets. Neuroreport 9(18):4119-22. [PubMed: 9926858]  [MGI Ref ID J:54045]

Baurle J; Helmchen C; Grusser-Cornehls U. 1997. Diverse effects of Purkinje cell loss on deep cerebellar and vestibular nuclei neurons in Purkinje cell degeneration mutant mice: a possible compensatory mechanism. J Comp Neurol 384(4):580-96. [PubMed: 9259491]  [MGI Ref ID J:42546]

Baurle J; Oestreicher AB; Gispen WH; Grusser-Cornehls U. 1994. Lesion-specific pattern of immunocytochemical distribution of growth-associated protein B-50 (GAP-43) in the cerebellum of Weaver and PCD-mutant mice: lack of B-50 involvement in neuroplasticity of Purkinje cell terminals? J Neurosci Res 38(3):327-35. [PubMed: 7932867]  [MGI Ref ID J:18698]

Baurle J; Vogten H; Grusser-Cornehls U. 1998. Course and targets of the calbindin D-28k subpopulation of primary vestibular afferents. J Comp Neurol 402(1):111-28. [PubMed: 9831049]  [MGI Ref ID J:118430]

Berrebi AS; Morgan JI; Mugnaini E. 1990. The Purkinje cell class may extend beyond the cerebellum. J Neurocytol 19(5):643-54. [PubMed: 2077109]  [MGI Ref ID J:121314]

Blanks JC; Mullen RJ; LaVail MM. 1982. Retinal degeneration in the pcd cerebellar mutant mouse. II. Electron microscopic analysis. J Comp Neurol 212(3):231-46. [PubMed: 7153375]  [MGI Ref ID J:6948]

Blanks JC; Spee C. 1992. Retinal degeneration in the pcd/pcd mutant mouse: accumulation of spherules in the interphotoreceptor space. Exp Eye Res 54(5):637-44. [PubMed: 1623950]  [MGI Ref ID J:12219]

Brown KL; Agelan A; Woodruff-Pak DS. 2010. Unimpaired trace classical eyeblink conditioning in Purkinje cell degeneration (pcd) mutant mice. Neurobiol Learn Mem 93(3):303-11. [PubMed: 19931625]  [MGI Ref ID J:166100]

Cabraja M; Baurle J. 2007. Vestibular ganglion neurons survive hair cell defects in jerker, shaker, and Varitint-waddler mutants and downregulate calretinin expression. J Comp Neurol 504(4):418-26. [PubMed: 17663432]  [MGI Ref ID J:132913]

Campbell DB; Hess EJ. 1996. Chromosomal localization of the neurological mouse mutations tottering (tg), Purkinje cell degeneration (pcd), and nervous (nr). Brain Res Mol Brain Res 37(1-2):79-84. [PubMed: 8738138]  [MGI Ref ID J:33012]

Campbell DB; North JB; Hess EJ. 1999. Tottering mouse motor dysfunction is abolished on the Purkinje cell degeneration (pcd) mutant background. Exp Neurol 160(1):268-78. [PubMed: 10630211]  [MGI Ref ID J:58539]

Chang AC; Ghetti B. 1993. Embryonic cerebellar graft development during acute phase of gliosis in the cerebellum of pcd mutant mice [published erratum appears in Chin J Physiol 1993;36(4):255] Chin J Physiol 36(3):141-9. [PubMed: 8194390]  [MGI Ref ID J:19591]

Chang B; Hawes NL; Hurd RE; Davisson MT; Nusinowitz S; Heckenlively JR. 2002. Retinal degeneration mutants in the mouse. Vision Res 42(4):517-25. [PubMed: 11853768]  [MGI Ref ID J:75095]

Chang B; Hawes NL; Hurd RE; Wang J; Howell D; Davisson MT; Roderick TH; Nusinowitz S; Heckenlively JR. 2005. Mouse models of ocular diseases. Vis Neurosci 22(5):587-93. [PubMed: 16332269]  [MGI Ref ID J:156373]

Chen L; Bao S; Lockard JM; Kim JK; Thompson RF. 1996. Impaired classical eyeblink conditioning in cerebellar-lesioned and Purkinje cell degeneration (pcd) mutant mice. J Neurosci 16(8):2829-38. [PubMed: 8786457]  [MGI Ref ID J:32197]

Chen L; Bao S; Thompson RF. 1999. Bilateral lesions of the interpositus nucleus completely prevent eyeblink conditioning in Purkinje cell-degeneration mutant mice. Behav Neurosci 113(1):204-10. [PubMed: 10197920]  [MGI Ref ID J:54145]

Chou DKH; Jungalwala FB. 1996. N-Acetylglucosaminyl transferase regulates the expression of the sulfoglucuronyl glycolipids in specific cell types in cerebellum during development. J Biol Chem 271(46):28868-74. [PubMed: 8910533]  [MGI Ref ID J:37189]

Delis F; Mitsacos A; Giompres P. 2004. Dopamine receptor and transporter levels are altered in the brain of Purkinje Cell Degeneration mutant mice. Neuroscience 125(1):255-68. [PubMed: 15051164]  [MGI Ref ID J:89977]

Doulazmi M; Hadj-Sahraoui N; Frederic F; Mariani J. 2002. Diminishing Purkinje cell populations in the cerebella of aging heterozygous Purkinje cell degeneration but not heterozygous nervous mice. J Neurogenet 16(2):111-23. [PubMed: 12479378]  [MGI Ref ID J:78159]

Fernandez-Gonzalez A; La Spada AR; Treadaway J; Higdon JC; Harris BS; Sidman RL; Morgan JI; Zuo J. 2002. Purkinje cell degeneration (pcd) phenotypes caused by mutations in the axotomy-induced gene, Nna1. Science 295(5561):1904-6. [PubMed: 11884758]  [MGI Ref ID J:74929]

Fujigasaki H; Song SY; Kobayashi T; Yamakuni T. 1996. Murine central neurons express a novel member of the cdc10/SWI6 motif-containing protein superfamily. Brain Res Mol Brain Res 40(2):203-13. [PubMed: 8872304]  [MGI Ref ID J:35715]

Gambarana C; Loria CJ; Siegel RE. 1993. GABAA receptor messenger RNA expression in the deep cerebellar nuclei of Purkinje cell degeneration mutants is maintained following the loss of innervating Purkinje neurons. Neuroscience 52(1):63-71. [PubMed: 8381926]  [MGI Ref ID J:21352]

Garin N; Hornung JP; Escher G. 2002. Distribution of postsynaptic GABA(A) receptor aggregates in the deep cerebellar nuclei of normal and mutant mice. J Comp Neurol 447(3):210-7. [PubMed: 11984816]  [MGI Ref ID J:77036]

Ghetti B; Alyea CJ; Muller J. 1978. Studies on the Purkinje Cell degeneration (pcd) mutant: Primary pathology and transneuronal changes J Neuropathol Exp Neurol 37:617 (Abstr 109).  [MGI Ref ID J:28476]

Ghetti B; Triarhou LC; Alyea CJ; Dlouhy SR; Karn RC. 1991. Unique cerebellar phenotype combining granule and Purkinje cell loss: morphological evidence for weaver* pcd double mutant mice. J Neurocytol 20(1):27-38. [PubMed: 2027034]  [MGI Ref ID J:121260]

Ghetti B; Triarhou LC; Fuller RW. 1993. Cerebellar Monoamines in the Purkinje Cell Degeneration Mutant Mouse. In: Serotonin, the Cerebellum, and Ataxia. Raven Press, New York.  [MGI Ref ID J:14367]

Gillardon F; Baurle J; Grusser-Cornehls U; Zimmermann M. 1995. DNA fragmentation and activation of c-Jun in the cerebellum of mutant mice (weaver, Purkinje cell degeneration). Neuroreport 6(13):1766-8. [PubMed: 8541477]  [MGI Ref ID J:29492]

Gillardon F; Baurle J; Wickert H; Grusser-Cornehls U; Zimmermann M. 1995. Differential regulation of bcl-2, bax, c-fos, junB, and krox-24 expression in the cerebellum of Purkinje cell degeneration mutant mice. J Neurosci Res 41(5):708-15. [PubMed: 7563251]  [MGI Ref ID J:27470]

Gomez C; Curto GG; Baltanas FC; Valero J; O'Shea E; Colado MI; Diaz D; Weruaga E; Alonso JR. 2012. Changes in the serotonergic system and in brain-derived neurotrophic factor distribution in the main olfactory bulb of pcd mice before and after mitral cell loss. Neuroscience 201:20-33. [PubMed: 22133893]  [MGI Ref ID J:184410]

Goodlett CR; Hamre KM; West JR. 1992. Dissociation of spatial navigation and visual guidance performance in Purkinje cell degeneration (pcd) mutant mice. Behav Brain Res 47(2):129-41. [PubMed: 1590945]  [MGI Ref ID J:1129]

Greer CA; Shepherd GM. 1982. Mitral cell degeneration and sensory function in the neurological mutant mouse Purkinje cell degeneration (PCD). Brain Res 235(1):156-61. [PubMed: 7188319]  [MGI Ref ID J:7473]

Handel MA; Dawson M. 1981. Effects on spermiogenesis in the mouse of a male sterile neurological mutation, Purkinje cell degeneration. Gamete Res 4:185-192.  [MGI Ref ID J:12097]

Hawes NL; Smith RS; Chang B; Davisson M; Heckenlively JR; John SW. 1999. Mouse fundus photography and angiography: a catalogue of normal and mutant phenotypes. Mol Vis 5:22. [PubMed: 10493779]  [MGI Ref ID J:59481]

Heckenlively JR; Chang B; Erway LC; Peng C; Hawes NL; Hageman GS; Roderick TH. 1995. Mouse model for Usher syndrome: linkage mapping suggests homology to Usher type I reported at human chromosome 11p15. Proc Natl Acad Sci U S A 92(24):11100-4. [PubMed: 7479945]  [MGI Ref ID J:121993]

Ikeda M; Morita I; Murota S; Sekiguchi F; Yuasa T; Miyatake T. 1993. Cerebellar nitric oxide synthase activity is reduced in nervous and Purkinje cell degeneration mutants but not in climbing fiber-lesioned mice. Neurosci Lett 155(2):148-50. [PubMed: 7690917]  [MGI Ref ID J:21353]

Jones BW; Watt CB; Frederick JM; Baehr W; Chen CK; Levine EM; Milam AH; Lavail MM; Marc RE. 2003. Retinal remodeling triggered by photoreceptor degenerations. J Comp Neurol 464(1):1-16. [PubMed: 12866125]  [MGI Ref ID J:84675]

Kambouris M; Sangameswaran L; Dlouhy SR; Hodes ME; Ghetti B; Triarhou LC. 1993. Cellular distribution of the RNA transcripts of a newly discovered gene in the brain of normal, weaver, Purkinje cell degeneration and reeler mutant mice as evidenced by in situ hybridization histochemistry. Brain Res Mol Brain Res 18(4):321-8. [PubMed: 8326827]  [MGI Ref ID J:11897]

Killian JE; Baker JF. 2002. Horizontal vestibuloocular reflex (VOR) head velocity estimation in Purkinje cell degeneration (pcd/pcd) mutant mice. J Neurophysiol 87(2):1159-64. [PubMed: 11826084]  [MGI Ref ID J:103198]

Kopmels B; Wollman EE; Guastavino JM; Delhaye-Bouchaud N; Fradelizi D; Mariani J. 1990. Interleukin-1 hyperproduction by in vitro activated peripheral macrophages from cerebellar mutant mice. J Neurochem 55(6):1980-5. [PubMed: 2230805]  [MGI Ref ID J:28095]

Kyuhou S; Gemba H. 2007. Fast cortical oscillation after thalamic degeneration: pivotal role of NMDA receptor. Biochem Biophys Res Commun 356(1):187-92. [PubMed: 17349613]  [MGI Ref ID J:121449]

Kyuhou S; Kato N; Gemba H. 2006. Emergence of endoplasmic reticulum stress and activated microglia in Purkinje cell degeneration mice. Neurosci Lett 396(2):91-6. [PubMed: 16356646]  [MGI Ref ID J:107944]

LaVail MM; Blanks JC; Mullen RJ. 1982. Retinal degeneration in the pcd cerebellar mutant mouse. I. Light microscopic and autoradiographic analysis. J Comp Neurol 212(3):217-30. [PubMed: 7153374]  [MGI Ref ID J:6947]

LaVail MM; Gorrin GM; Yasumura D; Matthes MT. 1999. Increased susceptibility to constant light in nr and pcd mice with inherited retinal degenerations. Invest Ophthalmol Vis Sci 40(5):1020-4. [PubMed: 10102304]  [MGI Ref ID J:53937]

LaVail MW; Yasumura D; Matthes MT; Lau-Villacorta C; Unoki K; Sung CH; Steinberg RH. 1998. Protection of mouse photoreceptors by survival factors in retinal degenerations. Invest Ophthalmol Vis Sci 39(3):592-602. [PubMed: 9501871]  [MGI Ref ID J:46230]

Lalonde R; Strazielle C. 2007. Spontaneous and induced mouse mutations with cerebellar dysfunctions: behavior and neurochemistry. Brain Res 1140:51-74. [PubMed: 16499884]  [MGI Ref ID J:120621]

Landis SC; Mullen RJ. 1978. The development and degeneration of Purkinje cells in pcd mutant mice. J Comp Neurol 177(1):125-43. [PubMed: 200636]  [MGI Ref ID J:5892]

Le Marec N; Lalonde R. 1997. Sensorimotor learning and retention during equilibrium tests in Purkinje cell degeneration mutant mice. Brain Res 768(1-2):310-6. [PubMed: 9369330]  [MGI Ref ID J:43580]

Luntz-Leybman V; Frostholm A; Fernando L; De Blas A; Rotter A. 1993. GABAA/benzodiazepine receptor gamma 2 subunit gene expression in developing normal and mutant mouse cerebellum. Brain Res Mol Brain Res 19(1-2):9-21. [PubMed: 8395631]  [MGI Ref ID J:12910]

Maeda N; Niinobe M; Inoue Y; Mikoshiba K. 1989. Developmental expression and intracellular location of P400 protein characteristic of Purkinje cells in the mouse cerebellum. Dev Biol 133(1):67-76. [PubMed: 2707487]  [MGI Ref ID J:16062]

Marchena M; Lara J; Aijon J; Germain F; de la Villa P; Velasco A. 2011. The retina of the PCD/PCD mouse as a model of photoreceptor degeneration. A structural and functional study. Exp Eye Res 93(5):607-17. [PubMed: 21824473]  [MGI Ref ID J:189268]

Matsuda K; Kondo T; Iijima T; Matsuda S; Watanabe M; Yuzaki M. 2009. Cbln1 binds to specific postsynaptic sites at parallel fiber-Purkinje cell synapses in the cerebellum. Eur J Neurosci 29(4):707-17. [PubMed: 19200061]  [MGI Ref ID J:146470]

Matsui K; Kato N; Watanabe N; Ando K. 1988. [Elevated immunoreactive-somatostatin levels in the brain of ataxic mutant mice] Jikken Dobutsu 37(3):263-8. [PubMed: 2901364]  [MGI Ref ID J:28478]

Matsui K; Masui A; Kato N; Adachi K. 1993. Levels of somatostatin and cholecystokinin in the brain of ataxic mutant mice. Life Sci 53(4):333-40. [PubMed: 8100981]  [MGI Ref ID J:14712]

Matsui K; Wada K; Kwak S. 1994. Ataxia-ameliorating effects of YM-14673, a potent analog of thyrotropin releasing hormone, in ataxic mutant mice. Eur J Pharmacol 254(3):295-7. [PubMed: 8013566]  [MGI Ref ID J:18435]

Milner TE; Cadoret G; Lessard L; Smith AM. 1995. EMG analysis of harmaline-induced tremor in normal and three strains of mutant mice with Purkinje cell degeneration and the role of the inferior olive. J Neurophysiol 73(6):2568-77. [PubMed: 7666163]  [MGI Ref ID J:29602]

Miret-Duvaux O; Frederic F; Simon D; Guenet JL; Hanauer A; Delhaye-Bouchaud N; Mariani J. 1990. Glutamate dehydrogenase in cerebellar mutant mice: gene localization and enzyme activity in different tissues. J Neurochem 54(1):23-9. [PubMed: 2293612]  [MGI Ref ID J:10148]

Mitsuma T; Adachi K; Mukoyama M; Ando K. 1990. Pro-thyrotropin-releasing hormone concentrations in the brain of ataxic mice. J Neurol Sci 98(2-3):163-7. [PubMed: 2123001]  [MGI Ref ID J:28467]

Mullen RJ. 1977. Site of pcd gene action and Purkinje cell mosaicism in cerebella of chimaeric mice. Nature 270(5634):245-7. [PubMed: 593342]  [MGI Ref ID J:5907]

Mullen RJ; Eicher EM; Sidman RL. 1976. Purkinje cell degeneration, a new neurological mutation in the mouse. Proc Natl Acad Sci U S A 73(1):208-12. [PubMed: 1061118]  [MGI Ref ID J:5613]

Mullen RJ; LaVail M. 1975. Two types of retinal degeneration in cerebellar mutant mice. Nature 258(5535):528-30. [PubMed: 1196386]  [MGI Ref ID J:5597]

O'Gorman S. 1985. Degeneration of thalamic neurons in Purkinje cell degeneration mutant mice. II. Cytology of neuron loss. J Comp Neurol 234(3):298-316. [PubMed: 3988986]  [MGI Ref ID J:7820]

O'Gorman S; Sidman RL. 1985. Degeneration of thalamic neurons in Purkinje cell degeneration mutant mice. I. Distribution of neuron loss. J Comp Neurol 234(3):277-97. [PubMed: 3988985]  [MGI Ref ID J:7819]

Roffler-Tarlov S; Landis SC; Zigmond MJ. 1984. Effects of Purkinje cell degeneration on the noradrenergic projection to mouse cerebellar cortex. Brain Res 298(2):303-11. [PubMed: 6144362]  [MGI Ref ID J:7434]

Rosenfeld JV; Richards LJ; Bartlett PF. 1993. Mutant mouse cerebellum does not provide specific signals for the selective migration and development of transplanted Purkinje cells. Neurosci Lett 155(1):19-23. [PubMed: 8361659]  [MGI Ref ID J:22001]

Rossi F; Jankovski A; Sotelo C. 1995. Target neuron controls the integrity of afferent axon phenotype: a study on the Purkinje cell-climbing fiber system in cerebellar mutant mice. J Neurosci 15(3 Pt 1):2040-56. [PubMed: 7891151]  [MGI Ref ID J:23733]

Rotter A; Rath S; Evans JE; Frostholm A. 2000. Modulation of GABA(A) receptor subunit mRNA levels in olivocerebellar neurons of purkinje cell degeneration and weaver mutant mice. J Neurochem 74(5):2190-200. [PubMed: 10800965]  [MGI Ref ID J:61537]

Ryo Y; Miyawaki A; Furuichi T; Mikoshiba K. 1993. Expression of the metabotropic glutamate receptor mGluR1 alpha and the ionotropic glutamate receptor GluR1 in the brain during the postnatal development of normal mouse and in the cerebellum from mutant mice. J Neurosci Res 36(1):19-32. [PubMed: 8230318]  [MGI Ref ID J:14425]

Sotelo C; Alvarado-Mallart RM. 1987. Embryonic and adult neurons interact to allow Purkinje cell replacement in mutant cerebellum. Nature 327(6121):421-3. [PubMed: 3587363]  [MGI Ref ID J:28470]

Sotelo C; Alvarado-Mallart RM. 1986. Growth and differentiation of cerebellar suspensions transplanted into the adult cerebellum of mice with heredodegenerative ataxia. Proc Natl Acad Sci U S A 83(4):1135-9. [PubMed: 3456566]  [MGI Ref ID J:28472]

Stasi K; Mitsacos A; Triarhou LC; Kouvelas ED. 1997. Cerebellar grafts partially reverse amino acid receptor changes observed in the cerebellum of mice with hereditary ataxia: quantitative autoradiographic studies. Cell Transplant 6(3):347-59. [PubMed: 9171167]  [MGI Ref ID J:41906]

Strazielle C; Lalonde R; Hebert C; Reader TA. 1999. Regional brain distribution of noradrenaline uptake sites, and of alpha1-alpha2- and beta-adrenergic receptors in PCD mutant mice: a quantitative autoradiographic study. Neuroscience 94(1):287-304. [PubMed: 10613519]  [MGI Ref ID J:118446]

Takeda H; Yoshiki A; Nishikawa S; Nishikawa S; Kunisada T; Sakakura T; Amanuma H; Kusakabe M. 1992. Expression of c-kit, a proto-oncogene of the murine W locus, in cerebella of normal and neurological mutant mice: immunohistochemical and in situ hybridization analysis. Differentiation 51(2):121-7. [PubMed: 1282111]  [MGI Ref ID J:2865]

Triarhou LC; Ghetti B. 1991. Serotonin-immunoreactivity in the cerebellum of two neurological mutant mice and the corresponding wild-type genetic stocks. J Chem Neuroanat 4(6):421-8. [PubMed: 1781951]  [MGI Ref ID J:805]

Triarhou LC; Low WC; Ghetti B. 1987. Transplantation of cerebellar anlagen to hosts with genetic cerebellocortical atrophy. Anat Embryol (Berl) 176(2):145-54. [PubMed: 3619071]  [MGI Ref ID J:28471]

Triarhou LC; Zhang W; Lee WH. 1996. Amelioration of the behavioral phenotype in genetically ataxic mice through bilateral intracerebellar grafting of fetal Purkinje cells. Cell Transplant 5(2):269-77. [PubMed: 8689037]  [MGI Ref ID J:32972]

Vaccarino FM; Ghetti B; Nurnberger JI Sr. 1985. Residual benzodiazepine (BZ) binding in the cortex of pcd mutant cerebella and qualitative BZ binding in the deep cerebellar nuclei of control and mutant mice: an autoradiographic study. Brain Res 343(1):70-8. [PubMed: 2994831]  [MGI Ref ID J:8014]

Valero J; Berciano MT; Weruaga E; Lafarga M; Alonso JR. 2006. Pre-neurodegeneration of mitral cells in the pcd mutant mouse is associated with DNA damage, transcriptional repression, and reorganization of nuclear speckles and Cajal bodies. Mol Cell Neurosci 33(3):283-95. [PubMed: 16978877]  [MGI Ref ID J:116561]

Varecka L; Wu CH; Rotter A; Frostholm A. 1994. GABAA/benzodiazepine receptor alpha 6 subunit mRNA in granule cells of the cerebellar cortex and cochlear nuclei: expression in developing and mutant mice. J Comp Neurol 339(3):341-52. [PubMed: 8132866]  [MGI Ref ID J:16213]

Wang T; Morgan JI. 2007. The Purkinje cell degeneration (pcd) mouse: an unexpected molecular link between neuronal degeneration and regeneration. Brain Res 1140:26-40. [PubMed: 16942761]  [MGI Ref ID J:120628]

Wassef M; Sotelo C; Cholley B; Brehier A; Thomasset M. 1987. Cerebellar mutations affecting the postnatal survival of Purkinje cells in the mouse disclose a longitudinal pattern of differentially sensitive cells. Dev Biol 124(2):379-89. [PubMed: 3678603]  [MGI Ref ID J:106414]

Won J; Shi LY; Hicks W; Wang J; Hurd R; Naggert JK; Chang B; Nishina PM. 2011. Mouse model resources for vision research. J Ophthalmol 2011:391384. [PubMed: 21052544]  [MGI Ref ID J:166679]

Zhang W; Ghetti B; Lee WH. 1997. Decreased IGF-I gene expression during the apoptosis of Purkinje cells in pcd mice. Brain Res Dev Brain Res 98(2):164-76. [PubMed: 9051257]  [MGI Ref ID J:38345]

Zhang W; Lee WH; Triarhou LC. 1996. Grafted cerebellar cells in a mouse model of hereditary ataxia express IGF-I system genes and partially restore behavioral function. Nat Med 2(1):65-71. [PubMed: 8564845]  [MGI Ref ID J:30905]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           FGB27

Colony Maintenance

Mating SystemOvarian Transplant-Cross-Intercross         (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 $232.00Female or MaleHomozygous for Agtpbp1pcd  
Price per Pair (US dollars $)Pair Genotype
$399.80Heterozygous for Agtpbp1pcd x Heterozygous for Agtpbp1pcd  

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.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $301.60Female or MaleHomozygous for Agtpbp1pcd  
Price per Pair (US dollars $)Pair Genotype
$519.80Heterozygous for Agtpbp1pcd x Heterozygous for Agtpbp1pcd  

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.

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.

General Supply Notes

  • View the complete collection of spontaneous mutants in the Mouse Mutant Resource.

Control Information

  Control
   Untyped from the colony
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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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.


See Terms of Use tab for General Terms and Conditions


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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Terms of Use


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phone:207-288-6470

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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