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| Mice that are homozygous for this Vps54 (vacuolar protein sorting 54 (yeast)) spontaneous mutation, wobbler, on the C57BL/6 background are viable, but infertile and die prematurely. Homozygotes exhibit progressive locomotor impairment with corresponding motor neuron and muscular degeneration. Homozygous males have defective spermiogenesis and are sterile. Homozygotes of both sexes have reduced serum estrogen. Mitochondria in motor neurons are abnormal. This mutant mouse strain may be useful in studies of Spinal Muscular Atrophy, Distal Hereditary Motor Neuronopathy and Amyotrophic Lateral Sclerosis 1. | |||||||||||||||
- Description
- Disease & phenotype
- Genes & alleles
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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; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Donating Investigator Michael Sendtner, University of Wuerzburg Description
Mice that are homozygous for this spontaneous mutation on the C57BL/6 background are viable, but infertile and die prematurely. The average lifespan is 3 months, although this can be increased if littermates are present in the cage, the Donating Investigator reports. Homozygotes exhibit progressive locomotor impairment with corresponding motor neuron and muscular degeneration. Homozygous males have defective spermiogenesis and are sterile. Homozygotes of both sexes have reduced serum estrogen. Mitochondria in motor neurons are abnormal. This mutant mouse strain may be useful in studies of Spinal Muscular Atrophy, Distal Hereditary Motor Neuronopathy and Amyotrophic Lateral Sclerosis 1.Development
The mutation was first recorded by Dr. D.S. Falconer at the Institute of Animal Genetics, Edinburgh, Scotland in 1956. The mutation arose in C57BL/Fa. In 1979 mice were obtained from Dr. Richard Sidman (The Childrens Hospital, Boston, MA, USA) by Dr. Harald Jockusch and backcrossed to C57BL/6J at University of Bielefeld (Bielefeld, Germany). In 2006 the strain was moved to Dr. Sendtner's laboratory at the University of Wuerzburg (Wuerzburg, Germany) and backcrossed to C57BL/6J for 7 generations.
Control Information
| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Related Strains
Amyotrophic Lateral Sclerosis (ALS)
010700 B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J 002298 B6.Cg-Tg(SOD1)2Gur/J 016149 B6.Cg-Tg(SOD1*G37R)1Dwc/J 008229 B6.Cg-Tg(SOD1*G37R)29Dpr/J 008342 B6.Cg-Tg(SOD1*G37R)42Dpr/J 008248 B6.Cg-Tg(SOD1*G85R)148Dwc/J 004435 B6.Cg-Tg(SOD1*G93A)1Gur/J 002299 B6.Cg-Tg(SOD1*G93A)dl1Gur/J 017907 B6N.Cg-Tg(Prnp-TARDBP)96Dwc/J 017933 B6N.Cg-Tg(Prnp-TARDBP*Q331K)103Dwc/J 017930 B6N.Cg-Tg(Prnp-TARDBP*Q331K)109Dwc/J 016201 B6SJL-Tg(Prnp-TARDBP)4Jlel/J 002297 B6SJL-Tg(SOD1)2Gur/J 002726 B6SJL-Tg(SOD1*G93A)1Gur/J 002300 B6SJL-Tg(SOD1*G93A)dl1Gur/J 016608 C57BL/6-Tg(Prnp-TARDBP)3cPtrc/J 017604 C57BL/6-Tg(Prnp-TARDBP*M337V)4Ptrc/J 002628 C57BL/6-Tg(SOD1)10Cje/J 002629 C57BL/6-Tg(SOD1)3Cje/J 005706 C57BL/6-Tg(tetO-CDK5R1/GFP)337Lht/J 008230 FVB(Cg)-Tg(Thy1-SOD1*G93A)T3Hgrd/J 005110 FVB-Tg(Sod1*G86R)M1Jwg/J 013199 FVB.Cg-Tg(SOD1*G93A)1Gur/J 013574 FVB/N-Tg(149m19)M141Kunst/J 017916 STOCK Tg(Prnp-FUS)WT3Cshw/J 016144 STOCK Tg(Prnp-TARDBP)4Jlel/J 016143 STOCK Tg(Prnp-TARDBP*A315T)23Jlel/J View Amyotrophic Lateral Sclerosis (ALS) (27 strains)
Additional Web Information
Reference Guide to Mouse Models of Spinal Muscular Atrophy manual [.pdf]
Working with ALS Mice manual [.pdf]
This resource was prepared by scientists with Prize4Life and The Jackson Laboratory.
Visit the Amyotrophic Lateral Sclerosis (ALS) Mouse Model Resource site for helpful information on ALS Disease and research resources.
Visit the Spinal Muscular Atrophy (SMA) Mouse Model Resource site for helpful information on SMA Disease and research resources.
Phenotype
Phenotype Information
- 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).
Amyotrophic Lateral Sclerosis 1; ALS1Models with phenotypic similarity to human diseases where etiology is unknown or involving genes where ortholog is unknown.
Spinal Muscular Atrophy, Type I; SMA1
assigned by genotype
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Vps54wr/Vps54wr
multiple strains
- mortality/aging
- premature death
- very reduced viability (MGI Ref ID J:165)
- growth/size phenotype
- decreased body weight (MGI Ref ID J:5102)
- behavior/neurological phenotype
- abnormal gait (MGI Ref ID J:6388)
- abnormal grip strength
- abnormal posture
- head and anterior trunk held lower than normal (MGI Ref ID J:5102)
- forelimb paralysis
- limb grasping (MGI Ref ID J:5102)
- unusual clasping of feet when suspended by tail (MGI Ref ID J:6388)
- tremors (MGI Ref ID J:165)
- fine tremors of the head (MGI Ref ID J:5102)
- nervous system phenotype
- abnormal nervous system morphology
- abnormal astrocyte morphology
- small numbers of GFAP positive cells in the anterior horn of the spinal cord at 1 month of age and found throughout the gray matter by 10 months (MGI Ref ID J:18368)
- astrocyte processes perpendicular to surface of cord rather than parallel as in controls (MGI Ref ID J:18368)
- GFAP reactive material gradually increases from 2 to 5 months of age (MGI Ref ID J:18368)
- abnormal motor neuron morphology
- motor neuron degeneration
- vacuolated neurons develop in the cervical spinal cord with much larger surface areas in x-section and smaller nuclear areas (MGI Ref ID J:6238)
- normal neurons are smaller in x-section but with normal nuclear areas (MGI Ref ID J:6238)
- protein synthesis reduced in both types of neurons (MGI Ref ID J:6238)
- vacuolar degeneration of motoneurons also in the lower brain stem (cerebellar, reticular, vestibular, cortical, and olfactory involvement variable) (MGI Ref ID J:6388)
- number of degenerating neurons increases with development of disease, none before onset of symptoms (MGI Ref ID J:19087)
- in brain stem and spinal cord but not basal ganglia or cerebral cortex (MGI Ref ID J:5102)
- progressive denervation of skeletal muscle (MGI Ref ID J:5102)
- sprouting from myelinated part of preterminal axons and nerve fibers innervate several muscle fibers (MGI Ref ID J:5102)
- reduced numbers of motor nerves in affected muscle (MGI Ref ID J:5102)
- abnormal spinal nerve morphology
- loss of large diameter nerve fibers in the median nerve (MGI Ref ID J:5331)
- abnormal sciatic nerve morphology
- loss of large diameter nerve fibers (MGI Ref ID J:5331)
- gliosis
- hypertrophy (MGI Ref ID J:1563)
- abnormal nervous system physiology (MGI Ref ID J:5525)
- abnormal CNS synaptic transmission
- levels of thyrotropin releasing hormone in the cervical spinal cord increase with progression of disease (MGI Ref ID J:3719)
- early increases in substance P in the hypothalamus, later increases in spinal cord and midbrain (MGI Ref ID J:3719)
- variable levels of Met- and Leu-enkephalin over the course of disease progression (MGI Ref ID J:3719)
- greater numbers of thyrotrophin releasing hormone neuronal processes in the ventrolateral horn of the spinal cord but decreasing with age (MGI Ref ID J:15226)
- reduced acetylcholinesterase containing cells in the ventral horn of the spinal cord (MGI Ref ID J:35070)
- substance P elevated in the ventral horn of the spinal cord early but becoming less as the disease progresses (MGI Ref ID J:35070)
- abnormal axonal transport
- abnormal myelination
- numbers of myelinated nerve fibers in the nerves to the arm and in the nerve to the tibialis anterior muscle were reduce 67-82% (MGI Ref ID J:5331)
- demyelination
- in conjunction with dissolution of axoplasm and nerve degeneration (MGI Ref ID J:5525)
- muscle phenotype
- abnormal muscle physiology (MGI Ref ID J:5102)
- abnormal skeletal muscle fiber morphology
- muscular atrophy
- neurogenic atrophy seen in muscles of mastication, neck, shoulder girdle, and intercostals (MGI Ref ID J:6388)
- lose of ability to extend paws at wrist (MGI Ref ID J:5102)
- atrophy of facial muscle gives a pointed appearance to the snout and results in the ears being laid back (MGI Ref ID J:5102)
- muscle atrophy seen by 6-7 weeks of age (MGI Ref ID J:5102)
- reproductive system phenotype
- abnormal gametogenesis (MGI Ref ID J:1563)
- asthenozoospermia
- sperm motility reduced to occasional trembling of the tail (MGI Ref ID J:1563)
- decreased testis weight
- testis weight 72% of normal (MGI Ref ID J:1563)
- infertility (MGI Ref ID J:6388)
- both sexes sterile (MGI Ref ID J:165)
- homeostasis/metabolism phenotype
- abnormal nucleotide metabolism
- cGMP levels decreased 80% in cervical spinal cord, 56% in the cerebellum, and 29% in the cortex (MGI Ref ID J:6029)
- decreased circulating estrogen level
- decreased levels in both sexes (MGI Ref ID J:6029)
- craniofacial phenotype
- upturned snout
- upward pointed snout (MGI Ref ID J:6388)
- liver/biliary system phenotype
- abnormal hepatocyte morphology
- vacuolar changes in hepatocytes (MGI Ref ID J:6388)
- cellular phenotype
- abnormal cell morphology
- all in degenerating neurons (MGI Ref ID J:5525)
- reduction and disorganization of rough endoplasmic reticulum (MGI Ref ID J:5525)
- ncrease in randomly organized neuronal cytoplasmic microtubules, filaments or smooth endoplasmic reticulum (MGI Ref ID J:5525)
- membrane bound dense bodies and /or lipid droplets (MGI Ref ID J:5525)
- abnormal lysosome morphology
- increased numbers of lysosomes and autophagic vacuoles (MGI Ref ID J:5525)
- endocrine/exocrine gland phenotype
- decreased testis weight
- testis weight 72% of normal (MGI Ref ID J:1563)
Vps54wr/Vps54wr
involves: C57BL/6J * C57BL/Fa
- nervous system phenotype
- abnormal GABA-mediated receptor currents
- mutant mice exhibit a down regulation in phasic and tonic GABA(a) receptor-mediated currents (MGI Ref ID J:181019)
- mice exhibit an 87% reduction in average tonic current densities (pA/pF) as compared to controls (MGI Ref ID J:181019)
- input resistance in layer 5 pyramidal neurons is increased in mutant mice as compared to controls (MGI Ref ID J:181019)
- current threshold is significantly lower in mutant mice, however polarization voltage is similar between mutant and control (MGI Ref ID J:181019)
- abnormal GABAergic neuron morphology
- decrease in density of parvalbumin-positive neurons in layer 5 of the primary motor cortex (MGI Ref ID J:181019)
- decrease in density of somatostatin-positive neurons in layer 5 of the primary motor cortex (MGI Ref ID J:181019)
- decrease in optical density of GABAergic synaptic boutons as assessed by VGAT staining in layer 5 of the primary motor cortex (MGI Ref ID J:181019)
- abnormal GABAergic neuron physiology
- layer 5 pyramidal neurons exhibit increased excitability as a result of decreased GABAergic inhibition (MGI Ref ID J:181019)
- abnormal inhibitory postsynaptic currents
- individual spontaneous inhibitory post synaptic currents (sIPSC) display smaller amplitudes, however mean amplitude is not statistically different in whole-cell voltage-clamp recordings from layer 5 pyramidal neurons (MGI Ref ID J:181019)
- sIPSC frequency is 28% lower than controls (MGI Ref ID J:181019)
- abnormal microglial cell activation
- microglia activation accompanies motor neuron degeneration (MGI Ref ID J:181019)
- abnormal miniature inhibitory postsynaptic currents
- abnormal synaptic bouton morphology
- decrease in optical density of GABAergic synaptic boutons as assessed by VGAT staining in layer 5 of the primary motor cortex (MGI Ref ID J:181019)
- gliosis
- reactive gliosis accompanies motor neuron degeneration (MGI Ref ID J:181019)
- motor neuron degeneration (MGI Ref ID J:181019)
- muscle phenotype
- progressive muscle weakness
- mice develop muscle weakness in the forelimbs beginning at 3-4 weeks (MGI Ref ID J:181019)
- immune system phenotype
- abnormal microglial cell activation
- microglia activation accompanies motor neuron degeneration (MGI Ref ID J:181019)
This mouse can be used to support research in many areas including:
Neurobiology Research
Amyotrophic Lateral Sclerosis (ALS)
Neurodegeneration
Spinal Muscular Atrophy (SMA)
Reproductive Biology Research
Fertility Defects
males only
Genes & Alleles
Gene & Allele Information provided by MGI
| Allele Symbol | Vps54wr | ||
|---|---|---|---|
| Allele Name | wobbler | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | wr; | ||
| Mutation Made By | Michael Sendtner, University of Wuerzburg | ||
| Strain of Origin | C57BL/Fa | ||
| Gene Symbol and Name | Vps54, vacuolar protein sorting 54 (yeast) | ||
| Chromosome | 11 | ||
| Gene Common Name(s) | 5330404P15Rik; HCC8; RIKEN cDNA 5330404P15 gene; SLP-8p; VPS54L; Vsp54; WR; hVps54L; mSLP8; wobbler; wr; | ||
| Molecular Note | An A to T transversion occurs in the second position of codon 967 in exon 23. This results in a leucine to glutamine amino acid substitution (L967Q). Gene splicing is not affected by this mutation. All three normal transcripts are detected. [MGI Ref ID J:104824] | ||
References
References provided by MGI
Selected Reference(s)
Falconer DS. 1956. Wobbler (wr) Mouse News Lett 15:23. [MGI Ref ID J:165]
Moser JM; Bigini P; Schmitt-John T. 2013. The wobbler mouse, an ALS animal model. Mol Genet Genomics 288(5-6):207-29. [PubMed: 23539154] [MGI Ref ID J:196432]
Additional References
Vps54wr relatedAit-Ikhlef A; Murawsky M; Blondet B; Hantaz-Ambroise D; Martinou JC; Rieger F. 1995. The motoneuron degeneration in the wobbler mouse is independent of the overexpression of a Bcl2 transgene in neurons. Neurosci Lett 199(3):163-6. [PubMed: 8577388] [MGI Ref ID J:30285]
Andrews JM. 1975. The fine structure of the cervical spinal cord, ventral root and brachial nerves in the wobbler (wr) mouse. J Neuropathol Exp Neurol 34(1):12-27. [PubMed: 1117319] [MGI Ref ID J:5525]
Augustin M; Heimann P; Rathke S; Jockusch H. 1997. Spinal muscular atrophy gene wobbler of the mouse: evidence from chimeric spinal cord and testis for cell-autonomous function. Dev Dyn 209(3):286-95. [PubMed: 9215643] [MGI Ref ID J:41765]
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]
Bartsch JW; Wildeboer D; Koller G; Naus S; Rittger A; Moss ML; Minai Y; Jockusch H. 2010. Tumor necrosis factor-alpha (TNF-alpha) regulates shedding of TNF-alpha receptor 1 by the metalloprotease-disintegrin ADAM8: evidence for a protease-regulated feedback loop in neuroprotection. J Neurosci 30(36):12210-8. [PubMed: 20826683] [MGI Ref ID J:164288]
Bigini P; Bastone A; Mennini T. 2001. Glutamate transporters in the spinal cord of the wobbler mouse. Neuroreport 12(9):1815-20. [PubMed: 11435904] [MGI Ref ID J:103640]
Blondet B; Ait-Ikhlef A; Murawsky M; Rieger F. 2001. Transient massive DNA fragmentation in nervous system during the early course of a murine neurodegenerative disease. Neurosci Lett 305(3):202-6. [PubMed: 11403940] [MGI Ref ID J:108043]
Blondet B; Barlovatz-Meimon G; Festoff BW; Soria C; Soria J; Rieger F; Hantai D. 1992. Plasminogen activators in the neuromuscular system of the wobbler mutant mouse. Brain Res 580(1-2):303-10. [PubMed: 1504807] [MGI Ref ID J:1302]
Blondet B; Carpentier G; Ait-Ikhlef A; Murawsky M; Rieger F. 2002. Motoneuron morphological alterations before and after the onset of the disease in the wobbler mouse. Brain Res 930(1-2):53-7. [PubMed: 11879795] [MGI Ref ID J:75616]
Blondet B; Hantaz-Ambroise D; Ait-Ikhlef A; Cambier D; Murawsky M; Rieger F. 1995. Astrocytosis in wobbler mouse spinal cord involves a population of astrocytes which is glutamine synthetase-negative. Neurosci Lett 183(3):179-82. [PubMed: 7739788] [MGI Ref ID J:23727]
Boillee S; Berruti G; Meccariello R; Grannec G; Razan F; Pierantoni R; Fasano S; Junier MP. 2002. Early defect in the expression of mouse sperm DNAJ 1, a member of the DNAJ/heat shock protein 40 chaperone protein family, in the spinal cord of the wobbler mouse, a murine model of motoneuronal degeneration. Neuroscience 113(4):825-35. [PubMed: 12182889] [MGI Ref ID J:120710]
Boillee S; Peschanski M; Junier MP. 2003. The wobbler mouse: a neurodegeneration jigsaw puzzle. Mol Neurobiol 28(1):65-106. [PubMed: 14514986] [MGI Ref ID J:197020]
Bose P; Fielding R; Ameis KM; Vacca-Galloway LL. 1998. A novel behavioral method to detect motoneuron disease in Wobbler mice aged three to seven days old. Brain Res 813(2):334-42. [PubMed: 9838183] [MGI Ref ID J:51273]
Bose P; Fielding R; Vacca-Galloway LL. 1999. Effects of assisted feeding on Wobbler mouse motoneuron disease and on serotonergic and peptidergic sprouting in the cervical spinal ventral horn. Brain Res Bull 48(4):429-39. [PubMed: 10357076] [MGI Ref ID J:55300]
Bose P; Vacca-Galloway LL. 1999. Increase in fiber density for immunoreactive serotonin, substance P, enkephalin and thyrotropin-releasing hormone occurs during the early presymptomatic period of motoneuron disease in Wobbler mouse spinal cord ventral horn. Neurosci Lett 260(3):196-200. [PubMed: 10076901] [MGI Ref ID J:53763]
Bronstein JM; Yamashita C; Farber DB. 1996. Exclusion of the beta-subunit of type II calmodulin kinase for the wobbler spinal muscular atrophy gene. Brain Res Mol Brain Res 43(1-2):330-2. [PubMed: 9037549] [MGI Ref ID J:37660]
Brooks BR; Lust WD; Andrews JM; Engel WK. 1978. Decreased spinal cord cGMP in murine (wobbler) spontaneous lower motor neuron degeneration. Arch Neurol 35(9):590-1. [PubMed: 210748] [MGI Ref ID J:6029]
Chelmicka-Schorr E; Sportiello M; Antel JP; Arnason BG. 1982. Acid protease activity in spinal cord and muscle in wobbler mouse. J Neurol Sci 56(2-3):141-5. [PubMed: 6757391] [MGI Ref ID J:6925]
Clowry GJ; McHanwell S. 2004. Brainstem motor nuclei respond differentially to degenerative disease in the mutant mouse wobbler. Neuropathol Appl Neurobiol 30(2):148-60. [PubMed: 15043712] [MGI Ref ID J:101795]
Clowry GJ; McHanwell S. 1996. Expression of nitric oxide synthase by motor neurones in the spinal cord of the mutant mouse wobbler. Neurosci Lett 215(3):177-80. [PubMed: 8899742] [MGI Ref ID J:36533]
Corvino V; Businaro R; Geloso MC; Bigini P; Cavallo V; Pompili E; Mennini T; Fumagalli L; Michetti F. 2003. S100B protein and 4-hydroxynonenal in the spinal cord of wobbler mice. Neurochem Res 28(2):341-5. [PubMed: 12608707] [MGI Ref ID J:106167]
Coulpier M; Junier MP; Peschanski M; Dreyfus PA. 1996. Bcl-2 sensitivity differentiates two pathways for motoneuronal death in the wobbler mutant mouse. J Neurosci 16(19):5897-904. [PubMed: 8815872] [MGI Ref ID J:35489]
Dave KR; Bradley WG; Perez-Pinzon MA. 2003. Early mitochondrial dysfunction occurs in motor cortex and spinal cord at the onset of disease in the Wobbler mouse. Exp Neurol 182(2):412-20. [PubMed: 12895451] [MGI Ref ID J:84853]
Dave KR; Raval AP; Purroy J; Kirkinezos IG; Moraes CT; Bradley WG; Perez-Pinzon MA. 2005. Aberrant deltaPKC activation in the spinal cord of Wobbler mouse: a model of motor neuron disease. Neurobiol Dis 18(1):126-33. [PubMed: 15649703] [MGI Ref ID J:95459]
Deng YP; Li XS; Zhang SH; Vacca-Galloway LL. 1996. Changes in receptor levels for thyrotropin releasing hormone, serotonin, and substance P in cervical spinal cord of Wobbler mouse: a quantitative autoradiography study during early and late stages of the motoneuron disease. Brain Res 725(1):49-60. [PubMed: 8828585] [MGI Ref ID J:34064]
Deniselle MC; Carreras MC; Garay L; Gargiulo-Monachelli G; Meyer M; Poderoso JJ; De Nicola AF. 2012. Progesterone prevents mitochondrial dysfunction in the spinal cord of wobbler mice. J Neurochem 122(1):185-95. [PubMed: 22486171] [MGI Ref ID J:186275]
Deniselle MC; Lavista-Llanos S; Ferrini MG; Lima AE; Roldan AG; De Nicola AF. 1999. In vitro differences between astrocytes of control and wobbler mice spinal cord. Neurochem Res 24(12):1535-41. [PubMed: 10591403] [MGI Ref ID J:106537]
Dennis JS; Citron BA. 2009. Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein. Neuroscience 158(2):745-50. [PubMed: 19013502] [MGI Ref ID J:145882]
Dockery P; Tang Y; Morais M; Vacca-Galloway LL. 1997. Neuron volume in the ventral horn in Wobbler mouse motoneuron disease: a light microscope stereological study. J Anat 191(Pt 1):89-98. [PubMed: 9279662] [MGI Ref ID J:42624]
Duchen LW; Strich SJ. 1968. An hereditary motor neurone disease with progressive denervation of muscle in the mouse: the mutant 'wobbler'. J Neurol Neurosurg Psychiatry 31(6):535-42. [PubMed: 5709840] [MGI Ref ID J:5102]
Eve DJ; Dennis JS; Citron BA. 2007. Transcription factor p53 in degenerating spinal cords. Brain Res 1150:174-81. [PubMed: 17434459] [MGI Ref ID J:122494]
Festoff BW; D'Andrea MR; Citron BA; Salcedo RM; Smirnova IV; Andrade-Gordon P. 2000. Motor neuron cell death in wobbler mutant mice follows overexpression of the G-protein-coupled, protease-activated receptor for thrombin. Mol Med 6(5):410-29. [PubMed: 10952021] [MGI Ref ID J:133832]
Fuchs S; Resch K; Thiel C; Ulbrich M; Platzer M; Jockusch H; Schmitt-John T. 2002. Comparative transcription map of the wobbler critical region on mouse chromosome 11 and the homologous region on human chromosome 2p13-14. BMC Genet 3(1):14. [PubMed: 12174196] [MGI Ref ID J:83520]
Fumagalli E; Bigini P; Barbera S; De Paola M; Mennini T. 2006. Riluzole, unlike the AMPA antagonist RPR119990, reduces motor impairment and partially prevents motoneuron death in the wobbler mouse, a model of neurodegenerative disease. Exp Neurol 198(1):114-28. [PubMed: 16386734] [MGI Ref ID J:107829]
Gonzalez Deniselle MC; Garay L; Gonzalez S; Guennoun R; Schumacher M; De Nicola AF. 2005. Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease. Exp Neurol 195(2):518-23. [PubMed: 16095593] [MGI Ref ID J:102543]
Gonzalez Deniselle MC; Garay L; Lopez-Costa JJ; Gonzalez S; Mougel A; Guennoun R; Schumacher M; De Nicola AF. 2004. Progesterone treatment reduces NADPH-diaphorase/nitric oxide synthase in Wobbler mouse motoneuron disease. Brain Res 1014(1-2):71-9. [PubMed: 15212993] [MGI Ref ID J:90944]
Gonzalez Deniselle MC; Gonzalez S; Piroli G; Ferrini M; Lima AE ; De Nicola AF. 1997. Glucocorticoid receptors and actions in the spinal cord of the Wobbler mouse, a model for neurodegenerative diseases. J Steroid Biochem Mol Biol 60(3-4):205-13. [PubMed: 9191978] [MGI Ref ID J:41051]
Gonzalez Deniselle MC; Gonzalez SL; Lima AE; Wilkin G; De Nicola AF. 1999. The 21-aminosteroid U-74389F attenuates hyperexpression of GAP-43 and NADPH-diaphorase in the spinal cord of wobbler mouse, a model for amyotrophic lateral sclerosis. Neurochem Res 24(1):1-8. [PubMed: 9973230] [MGI Ref ID J:53014]
Haenggeli C; Kato AC. 2002. Differential vulnerability of cranial motoneurons in mouse models with motor neuron degeneration. Neurosci Lett 335(1):39-43. [PubMed: 12457737] [MGI Ref ID J:131493]
Hantai D; Akaaboune M; Lagord C; Murawsky M; Houenou LJ; Festoff BW; Vaught JL; Rieger F; Blondet B. 1995. Beneficial effects of insulin-like growth factor-I on wobbler mouse motoneuron disease. J Neurol Sci 129 Suppl:122-6. [PubMed: 7595602] [MGI Ref ID J:30388]
Hantaz-Ambroise D; Blondet B; Murawsky M; Rieger F. 1994. Abnormal astrocyte differentiation and defective cellular interactions in wobbler mouse spinal cord. J Neurocytol 23(3):179-92. [PubMed: 8006678] [MGI Ref ID J:18368]
Hantaz-Ambroise D; Jacque C; Ait Ikhlef A; Parmentier C; Leclerc P; Cambier D; Zadigue G; Rieger F. 2001. Specific features of chronic astrocyte gliosis after experimental central nervous system (CNS) xenografting and in Wobbler neurological mutant CNS. Differentiation 69(2-3):100-7. [PubMed: 11798064] [MGI Ref ID J:73814]
Heimann P; Laage S; Jockusch H. 1991. Defect of sperm assembly in a neurological mutant of the mouse, wobbler (WR). Differentiation 47(2):77-83. [PubMed: 1955109] [MGI Ref ID J:1563]
Ikeda K; Iwasaki Y; Kinoshita M. 1998. Neuronal nitric oxide synthase inhibitor, 7-nitroindazole, delays motor dysfunction and spinal motoneuron degeneration in the wobbler mouse. J Neurol Sci 160(1):9-15. [PubMed: 9804111] [MGI Ref ID J:53630]
Ikeda K; Iwasaki Y; Kinoshita M; Marubuchi S; Ono S. 2000. T-588, a novel neuroprotective agent, delays progression of neuromuscular dysfunction in wobbler mouse motoneuron disease. Brain Res 858(1):84-91. [PubMed: 10700601] [MGI Ref ID J:60779]
Ikeda K; Iwasaki Y; Tagaya N; Shiojima T; Kinoshita M. 1995. Neuroprotective effect of cholinergic differentiation factor/leukemia inhibitory factor on wobbler murine motor neuron disease. Muscle Nerve 18(11):1344-7. [PubMed: 7565937] [MGI Ref ID J:30224]
Ikeda K; Iwasaki Y; Tagaya N; Shiojima T; Kobayashi T; Kinoshita M. 1995. Neuroprotective effect of basic fibroblast growth factor on wobbler mouse motor neuron disease. Neurol Res 17(6):445-8. [PubMed: 8622800] [MGI Ref ID J:33893]
Ikeda K; Kinoshita M; Iwasaki Y. 1996. Basic fibroblast growth factor has neuroprotective effects on axotomy-induced spinal motoneuron death and wobbler mouse motoneuron disease [letter] [see comments] Muscle Nerve 19(6):794-5. [PubMed: 8609937] [MGI Ref ID J:33898]
Ikeda K; Kinoshita M; Iwasaki Y; Tagaya N; Shiojima T. 1995. Lecithinized superoxide dismutase retards wobbler mouse motoneuron disease. Neuromuscul Disord 5(5):383-90. [PubMed: 7496172] [MGI Ref ID J:31054]
Ikeda K; Kinoshita M; Tagaya N; Shiojima T; Taga T; Yasukawa K; Suzuki H; Okano A. 1996. Coadministration of interleukin-6 (IL-6) and soluble IL-6 receptor delays progression of wobbler mouse motor neuron disease. Brain Res 726(1-2):91-7. [PubMed: 8836549] [MGI Ref ID J:34353]
Ikeda K; Klinkosz B; Greene T; Cedarbaum JM; Wong V; Lindsay RM; Mitsumoto H. 1995. Effects of brain-derived neurotrophic factor on motor dysfunction in wobbler mouse motor neuron disease. Ann Neurol 37(4):505-11. [PubMed: 7717687] [MGI Ref ID J:25723]
Ikeda K; Mitsumoto H. 1993. In vivo and in vitro muscle tensions in wobbler mouse motor neuron disease [letter] Muscle Nerve 16(9):979-81. [PubMed: 8355733] [MGI Ref ID J:15582]
Ikeda K; Wong V; Holmlund TH; Greene T; Cedarbaum JM; Lindsay RM; Mitsumoto H. 1995. Histometric effects of ciliary neurotrophic factor in wobbler mouse motor neuron disease. Ann Neurol 37(1):47-54. [PubMed: 7818257] [MGI Ref ID J:23028]
Ishiyama T; Klinkosz B; Pioro EP; Mitsumoto H. 1997. Genetic transfer of the wobbler gene to a C57BL/6J x NZB hybrid stock: natural history of the motor neuron disease and response to CNTF and BDNF cotreatment. Exp Neurol 148(1):247-55. [PubMed: 9398466] [MGI Ref ID J:44422]
Jockusch H; Laage S; Kaupmann K; Heimann P. 1991. Defective Sperm Assembly in a Neurological Mutant of the Mouse, Wobbler. In: Comparative Spermatology 20 Years After. Raven Press, New York. [MGI Ref ID J:3631]
Junier MP; Coulpier M; Le Forestier N; Cadusseau J; Suzuki F; Peschanski M; Dreyfus PA. 1994. Transforming growth factor alpha (TGF alpha) expression in degenerating motoneurons of the murine mutant wobbler: a neuronal signal for astrogliosis? J Neurosci 14(7):4206-16. [PubMed: 8027772] [MGI Ref ID J:19087]
Junier MP; Legendre P; Esguerra CV; Tinel M; Coulpier M; Dreyfus PA ; Bahr M. 1998. Regulation of growth factor gene expression in degenerating motoneurons of the murine mutant wobbler: a cellular patch-sampling/RT-PCR study. Mol Cell Neurosci 12(3):168-77. [PubMed: 9790737] [MGI Ref ID J:50867]
Kaupmann K; Sendtner M; Stockli KA; Jockusch H. 1991. The gene for ciliary neurotrophic factor (CNTF) maps to murine Chromosome 19 and its expression is not affected in the hereditary motoneuron disease 'Wobbler' of the mouse Eur J Neurosci 3:1182-86. [PubMed: 12106247] [MGI Ref ID J:14942]
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]
Krieger C; Perry TL; Hansen S; Mitsumoto H; Honore T. 1992. Excitatory amino acid receptor antagonist in murine motoneuron disease (the wobbler mouse). Can J Neurol Sci 19(4):462-5. [PubMed: 1423043] [MGI Ref ID J:3860]
Laage S; Zobel G; Jockusch H. 1988. Astrocyte overgrowth in the brain stem and spinal cord of mice affected by spinal atrophy, wobbler. Dev Neurosci 10(3):190-8. [PubMed: 3142758] [MGI Ref ID J:35069]
Leestma JE. 1980. Animal model of human disease: Werdnig-Hoffmann disease (infantile spinal muscular atrophy). Am J Pathol 100(3):821-4. [PubMed: 7416238] [MGI Ref ID J:6388]
Ma WY; Vacca-Galloway LL. 1991. Reduced branching and length of dendrites detected in cervical spinal cord motoneurons of Wobbler mouse, a model for inherited motoneuron disease. J Comp Neurol 311(2):210-22. [PubMed: 1721631] [MGI Ref ID J:2008]
Ma WY; Vaccagalloway LL. 1992. Spiny interneurons identified in the normal mouse spinal cord show alterations in the Wobbler mouse - A model for inherited motoneuron disease Restorative Neurol Neurosci 4(6):381-392. [MGI Ref ID J:4095]
Meccariello R; Cobellis G; Berruti G; Junier MP; Ceriani M; Boilee S; Pierantoni R; Fasano S. 2002. Mouse sperm cell-specific DnaJ first homologue: an evolutionarily conserved protein for spermiogenesis. Biol Reprod 66(5):1328-35. [PubMed: 11967194] [MGI Ref ID J:108649]
Meyer M; Gonzalez Deniselle MC; Gargiulo-Monachelli G; Garay LI; Schumacher M; Guennoun R; De Nicola AF. 2012. Progesterone effects on neuronal brain-derived neurotrophic factor and glial cells during progression of Wobbler mouse neurodegeneration. Neuroscience 201:267-79. [PubMed: 22123169] [MGI Ref ID J:184416]
Mitsumoto H; Gambetti P. 1986. Impaired slow axonal transport in wobbler mouse motor neuron disease. Ann Neurol 19(1):36-43. [PubMed: 3947038] [MGI Ref ID J:8186]
Mitsumoto H; Ikeda K; Holmlund T; Greene T; Cedarbaum JM; Wong V; Lindsay RM. 1994. The effects of ciliary neurotrophic factor on motor dysfunction in wobbler mouse motor neuron disease [see comments] Ann Neurol 36(2):142-8. [PubMed: 8053649] [MGI Ref ID J:20393]
Mitsumoto H; Ikeda K; Klinkosz B; Cedarbaum JM; Wong V; Lindsay RM. 1994. Arrest of motor neuron disease in wobbler mice cotreated with CNTF and BDNF [see comments] Science 265(5175):1107-10. [PubMed: 8066451] [MGI Ref ID J:20053]
Mitsumoto H; Kurahashi K; Jacob JM; McQuarrie IG. 1993. Retardation of fast axonal transport in wobbler mice. Muscle Nerve 16(5):542-7. [PubMed: 8390608] [MGI Ref ID J:14361]
Murakami T; Mastaglia FL; Bradley WG. 1980. Reduced protein synthesis in spinal anterior horn neurons in wobbler mouse mutant. Exp Neurol 67(2):423-32. [PubMed: 7349996] [MGI Ref ID J:6238]
Nieto-Gonzalez JL; Moser J; Lauritzen M; Schmitt-John T; Jensen K. 2011. Reduced GABAergic inhibition explains cortical hyperexcitability in the wobbler mouse model of ALS. Cereb Cortex 21(3):625-35. [PubMed: 20643756] [MGI Ref ID J:181019]
Perez-Victoria FJ; Abascal-Palacios G; Tascon I; Kajava A; Magadan JG; Pioro EP; Bonifacino JS; Hierro A. 2010. Structural basis for the wobbler mouse neurodegenerative disorder caused by mutation in the Vps54 subunit of the GARP complex. Proc Natl Acad Sci U S A 107(29):12860-5. [PubMed: 20615984] [MGI Ref ID J:162305]
Pernas-Alonso R; Perrone-Capano C; Volpicelli F; di Porzio U. 2001. Regionalized neurofilament accumulation and motoneuron degeneration are linked phenotypes in wobbler neuromuscular disease. Neurobiol Dis 8(4):581-9. [PubMed: 11493023] [MGI Ref ID J:71188]
Pernas-Alonso R; Schaffner AE; Hansen CT; Barker JL; di Porzio U. 1995. Acetylcholine esterase and peripherin mRNA level decrease in wobbler mouse. Neuroreport 6(4):597-600. [PubMed: 7605908] [MGI Ref ID J:25043]
Pernas-Alonso R; Schaffner AE; Perrone-Capano C; Orlando A; Morelli F; Hansen CT; Barker JL; Esposito B; Cacucci F; di Porzio U. 1996. Early upregulation of medium neurofilament gene expression in developing spinal cord of the wobbler mouse mutant. Brain Res Mol Brain Res 38(2):267-75. [PubMed: 8793115] [MGI Ref ID J:33482]
Perrin FE; Boisset G; Lathuiliere A; Kato AC. 2006. Cell death pathways differ in several mouse models with motoneurone disease: analysis of pure motoneurone populations at a presymptomatic age. J Neurochem 98(6):1959-72. [PubMed: 16831193] [MGI Ref ID J:112591]
Pioro EP; Wang Y; Moore JK; Ng TC; Trapp BD; Klinkosz B; Mitsumoto H. 1998. Neuronal pathology in the wobbler mouse brain revealed by in vivo proton magnetic resonance spectroscopy and immunocytochemistry. Neuroreport 9(13):3041-6. [PubMed: 9804313] [MGI Ref ID J:103720]
Pollin MM; McHanwell S; Slater CR. 1990. Loss of motor neurons from the median nerve motor nucleus of the mutant mouse 'wobbler'. J Neurocytol 19(1):29-38. [PubMed: 2351995] [MGI Ref ID J:121315]
Popper P; Farber DB; Micevych PE; Minoofar K; Bronstein JM. 1997. TRPM-2 expression and tunel staining in neurodegenerative diseases: studies in wobbler and rd mice. Exp Neurol 143(2):246-54. [PubMed: 9056387] [MGI Ref ID J:38831]
Rathke-Hartlieb S; Budde P; Ewert S; Schlomann U; Staege MS; Jockusch H; Bartsch JW; Frey J. 2000. Elevated expression of membrane type 1 metalloproteinase (MT1-MMP) in reactive astrocytes following neurodegeneration in mouse central nervous system. FEBS Lett 481(3):227-34. [PubMed: 11007969] [MGI Ref ID J:115115]
Rathke-Hartlieb S; Schmidt VC; Jockusch H; Schmitt-John T; Bartsch JW. 1999. Spatiotemporal progression of neurodegeneration and glia activation in the wobbler neuropathy of the mouse Neuroreport 10(16):3411-6. [PubMed: 10599854] [MGI Ref ID J:59810]
Santoro B; Bigini P; Levandis G; Nobile V; Biggiogera M; Botti F; Mennini T; Curti D. 2004. Evidence for chronic mitochondrial impairment in the cervical spinal cord of a murine model of motor neuron disease. Neurobiol Dis 17(2):349-57. [PubMed: 15474372] [MGI Ref ID J:93089]
Schlomann U; Rathke-Hartlieb S; Yamamoto S; Jockusch H; Bartsch JW. 2000. Tumor necrosis factor alpha induces a metalloprotease-disintegrin, ADAM8 (CD 156): implications for neuron-glia interactions during neurodegeneration J Neurosci 20(21):7964-71. [PubMed: 11050116] [MGI Ref ID J:65181]
Schmitt-John T; Drepper C; Mussmann A; Hahn P; Kuhlmann M; Thiel C; Hafner M; Lengeling A; Heimann P; Jones JM; Meisler MH; Jockusch H. 2005. Mutation of Vps54 causes motor neuron disease and defective spermiogenesis in the wobbler mouse. Nat Genet 37(11):1213-5. [PubMed: 16244655] [MGI Ref ID J:104824]
Sedehizade F; Klocke R; Jockusch H. 1997. Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies. Muscle Nerve 20(2):186-94. [PubMed: 9040657] [MGI Ref ID J:53205]
Shi J; Vacca-Galloway LL. 1993. Thyrotropin-releasing hormone (TRH) neurons sprout in cervical spinal cord of Wobbler mouse. Brain Res 626(1-2):83-9. [PubMed: 8281455] [MGI Ref ID J:15226]
Smith ME; Hughes S. 1994. POMC neuropeptides and their receptors in the neuromuscular system of wobbler mice. J Neurol Sci 124 Suppl:56-8. [PubMed: 7807142] [MGI Ref ID J:35068]
Staunton L; Jockusch H; Ohlendieck K. 2011. Proteomic analysis of muscle affected by motor neuron degeneration: the wobbler mouse model of amyotrophic lateral sclerosis. Biochem Biophys Res Commun 406(4):595-600. [PubMed: 21354103] [MGI Ref ID J:171954]
Stuhlfauth I; Reininghaus J; Jockusch H; Heizmann CW. 1984. Calcium-binding protein, parvalbumin, is reduced in mutant mammalian muscle with abnormal contractile properties. Proc Natl Acad Sci U S A 81(15):4814-8. [PubMed: 6589628] [MGI Ref ID J:7524]
Tomiyama M; Kannari K; Nunomura J; Oyama Y; Takebe K; Matsunaga M. 1994. Quantitative autoradiographic distribution of glutamate receptors in the cervical segment of the spinal cord of the wobbler mouse. Brain Res 650(2):353-7. [PubMed: 7953705] [MGI Ref ID J:19164]
Toursel T; Bastide B; Stevens L; Rieger F; Mounier Y. 2000. Alterations in contractile properties and expression of myofibrillar proteins in wobbler mouse muscles. Exp Neurol 162(2):311-20. [PubMed: 10739637] [MGI Ref ID J:115319]
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Vergani L; Finco C; Di Giulio AM; Muller EE; Gorio A. 1997. Effects of low doses of glycosaminoglycans and insulin-like growth factor-I on motor neuron disease in wobbler mouse. Neurosci Lett 228(1):41-4. [PubMed: 9197283] [MGI Ref ID J:57351]
Xu GP; Dave KR; Moraes CT; Busto R; Sick TJ; Bradley WG; Perez-Pinzon MA. 2001. Dysfunctional mitochondrial respiration in the wobbler mouse brain. Neurosci Lett 300(3):141-4. [PubMed: 11226631] [MGI Ref ID J:107929]
Yung KK; Tang F; Fielding R; Du YH; Vacca-Galloway LL. 1992. Alteration in the levels of thyrotropin releasing hormone, substance P and enkephalins in the spinal cord, brainstem, hypothalamus and midbrain of the Wobbler mouse at different stages of the motoneuron disease [published erratum appears in Neurosciecne993 Aug;55(3):881] Neuroscience 50(1):209-22. [PubMed: 1383870] [MGI Ref ID J:3719]
Yung KK; Tang F; Vacca-Galloway LL. 1994. Alterations in acetylcholinesterase and choline acetyltransferase activities and neuropeptide levels in the ventral spinal cord of the Wobbler mouse during inherited motoneuron disease. Brain Res 638(1-2):337-42. [PubMed: 7515324] [MGI Ref ID J:17037]
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Health & husbandry
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.
Health & Colony Maintenance Information
Animal Health Reports
Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.Colony Maintenance
Breeding & Husbandry When maintaining a live colony, these mice can be bred as heterozygotes. Homozygotes are viable but are infertile and die prematurely.
Pricing and Purchasing
Pricing, Supply Level & Notes, Controls
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2085.00 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.
Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
Supply Notes
Cryorecovery - Standard.
Progeny testing is not required.
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 11 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.Cryorecovery to establish a Dedicated Supply for greater quantities of mice
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2710.50 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.
Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
Supply Notes
Cryorecovery - Standard.
Progeny testing is not required.
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 11 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.Cryorecovery to establish a Dedicated Supply for greater quantities of mice
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
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Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
General Supply Notes
- View the complete collection of spontaneous mutants in the Mouse Mutant Resource.
Control Information
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| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
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| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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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.Ordering Information
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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
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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.