Strain Name:

B6.B-Vps54wr/J

Stock Number:

009680

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Availability:

Cryopreserved - Ready for recovery

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

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.
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Additional information on Congenic nomenclature.
Specieslaboratory 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

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Spinal Muscular Atrophy (SMA) Models
008849   B6.129(C)-Smn1tm1.1Jme/J
006146   B6.129-Smn1tm1Jme/J
008453   B6.129-Smn1tm4(SMN2)Mrph/J
008714   B6.129-Smn1tm5(Smn1/SMN2)Mrph/J
009378   B6.129-Smn1tm6(SMN2)Mrph/J
010921   B6.129P2(Cg)-Smn1tm1Msd/J
018439   B6.129S6-Tg(CAG-Bgeo,-SMN2)E9Dscd/J
007963   B6.Cg-Smn1tm2Mrph/J
007966   B6.Cg-Smn1tm3(SMN2/Smn1)Mrph/J
006149   B6.Cg-Tg(ACTA1-cre)79Jme/J
006663   B6.Cg-Tg(Eno2-cre)39Jme/J
008629   B6.Cg-Tg(SMN2)11Tro Smn1tm1Msd/J
008631   B6.Cg-Tg(SMN2)11Tro Tg(SMN2)46Tro Smn1tm1Msd/J
008630   B6.Cg-Tg(SMN2)46Tro Smn1tm1Msd/J
007222   B6.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN1*A2G)2023Ahmb/J
006964   B6.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN2*delta7)4299Ahmb/J
006773   B6.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd/J
007246   B6;129-Smn1tm2Mrph/J
008383   B6;129-Smn1tm4(SMN2)Mrph/J
008384   B6;129-Smn1tm5(Smn1/SMN2)Mrph/J
008704   B6;129-Smn1tm6(SMN2)Mrph/J
006138   FVB.129(B6)-Smn1tm1Jme/J
008713   FVB.129(B6)-Smn1tm4(SMN2)Mrph/J
008604   FVB.129(B6)-Smn1tm5(Smn1/SMN2)Mrph/J
006214   FVB.129P2-Smn1tm1Msd/J
005058   FVB.Cg-Smn1tm1Hung Tg(SMN2)2Hung/J
008206   FVB.Cg-Smn1tm1Msd Tg(SMN2)566Ahmb/J
007955   FVB.Cg-Smn1tm2Mrph/J
007964   FVB.Cg-Smn1tm3(SMN2/Smn1)Mrph/J
009381   FVB.Cg-Smn1tm6(SMN2)Mrph/J
012252   FVB.Cg-Tbcepmn/J
006139   FVB.Cg-Tg(ACTA1-cre)79Jme/J
006297   FVB.Cg-Tg(Eno2-cre)39Jme/J
008209   FVB.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd Tg(ACTA1-SMN)69Ahmb/J
016573   FVB.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd Tg(S100B-EGFP)1Wjt Tg(SMN2*delta7)4299Ahmb/J
007968   FVB.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN1*A2G)2023Ahmb/2J
008782   FVB.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN2*A111G)588Ahmb/J
009134   FVB.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN2*A111G)591Ahmb/J
007952   FVB.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN2*delta7)4299Ahmb/2J
005025   FVB.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN2*delta7)4299Ahmb/J
005026   FVB.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN1*A2G)2023Ahmb/J
007949   FVB.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd/2J
005024   FVB.Cg-Tg(SMN2)89Ahmb Smn1tm1Msd/J
009682   NMRI-Tbcepmn/J
017596   STOCK Gt(ROSA)26Sortm1.1(rtTA,EGFP)Nagy Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN2*delta7)4299Ahmb Tg(tetO-SMN2,-luc)#aAhmb/J
017597   STOCK Gt(ROSA)26Sortm1.1(rtTA,EGFP)Nagy Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN2*delta7)4299Ahmb Tg(tetO-SMN2,-luc)#bAhmb/J
007022   STOCK Mnx1tm4(cre)Tmj Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN2*delta7)4299Ahmb/J
008783   STOCK Tg(CAG-cre/Esr1*)5Amc Smn1tm3(SMN2/Smn1)Mrph Tg(SMN2*delta7)4299Ahmb Tg(SMN2)89Ahmb/J
005938   STOCK Tg(Eno2-cre)39Jme/J
008203   STOCK Tg(SMN2)89Ahmb Smn1tm1Msd Tg(ACTA1-SMN)63Ahmb/J
006553   STOCK Tg(SMN2)89Ahmb Smn1tm1Msd Tg(H2-K1-tsA58)6Kio Tg(SMN2*delta7)4299Ahmb/J
006570   STOCK Tg(SMN2)89Ahmb Smn1tm1Msd Tg(Hlxb9-GFP)1Tmj/J
008212   STOCK Tg(SMN2)89Ahmb Smn1tm1Msd Tg(Prnp-SMN)92Ahmb/J
018916   STOCK Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN1-SMN2*)16Cll/CllJ
007951   STOCK Tg(SMN2)89Ahmb Smn1tm3(SMN2/Smn1)Mrph Tg(SMN2*delta7)4299Ahmb/J
008359   STOCK Tg(SMN2)89Ahmb Smn1tm3(SMN2/Smn1)Mrph/J
017599   STOCK Tg(tetO-SMN2,-luc)#aAhmb/J
017600   STOCK Tg(tetO-SMN2,-luc)#bAhmb/J
View Spinal Muscular Atrophy (SMA) Models     (58 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

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- 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; ALS1
Spinal Muscular Atrophy, Type I; SMA1
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.

Vps54wr/Vps54wr

        multiple strains
  • mortality/aging
  • premature death
    • very reduced viability   (MGI Ref ID J:165)
  • growth/size/body phenotype
  • decreased body weight   (MGI Ref ID J:5102)
    • at 30 days of age   (MGI Ref ID J:1563)
    • 47% normal   (MGI Ref ID J:1563)
  • upturned snout
    • upward pointed snout   (MGI Ref ID J:6388)
  • behavior/neurological phenotype
  • abnormal gait   (MGI Ref ID J:6388)
    • by 30 days of age   (MGI Ref ID J:1563)
    • high stepping and unsteady   (MGI Ref ID J:5102)
    • eventually walk on dorsum of forepaws due to the inability to extend the paws at the wrist   (MGI Ref ID J:5102)
    • ultimately they push the body along using their hind legs only   (MGI Ref ID J:5102)
  • abnormal grip strength
    • in forepaws   (MGI Ref ID J:5102)
    • muscle weakness by fourth or fifth week   (MGI Ref ID J:5102)
  • abnormal posture
    • head and anterior trunk held lower than normal   (MGI Ref ID J:5102)
  • forelimb paralysis
    • paralysis primarily affects forelimbs   (MGI Ref ID J:165)
    • clasping of front paws and difficulties using front legs   (MGI Ref ID J:1563)
  • 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
    • brain stem and spinal cord through the thoracic region weighs 62-68% of controls   (MGI Ref ID J:35069)
    • cross section area of spinal cord 75-80% of normal   (MGI Ref ID J:35069)
    • 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
      • enlarged soma   (MGI Ref ID J:19087)
      • poorly stained Nissl bodies   (MGI Ref ID J:19087)
      • reduced numbers of dendrites   (MGI Ref ID J:19087)
      • eccentric nuclei   (MGI Ref ID J:5102)
      • 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
      • fast axonal transport rate reduced 25%   (MGI Ref ID J:14361)
      • impaired slow axonal transport affecting neurofilament proteins more than tubulin and actin   (MGI Ref ID J:8186)
    • 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)
    • muscle weakness
      • by fourth or fifth week   (MGI Ref ID J:5102)
      • particularly involves forelimbs   (MGI Ref ID J:5102)
  • abnormal skeletal muscle fiber morphology
    • enlargement of sarcolemmal nuclei which migrate to the center of the muscle fiber   (MGI Ref ID J:5102)
    • muscle fibers eventually decrease in diameter   (MGI Ref ID J:5102)
    • deposition of fat between atrophied muscle fibers   (MGI Ref ID J:5102)
  • 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)
    • globozoospermia
      • lack of intact acrosome   (MGI Ref ID J:1563)
      • round sperm heads   (MGI Ref ID J:1563)
    • oligozoospermia
      • sperm numbers 70-80% of controls   (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
    • whole cell recordings of miniature IPSC (mIPSC) in layer 5 pyramidal neurons detect an increase in weighted decay time constant, but no differences in amplitude or rise time   (MGI Ref ID J:181019)
    • mIPSC frequency is 36% lower than controls   (MGI Ref ID J:181019)
  • 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)
  • hematopoietic system phenotype
  • abnormal microglial cell activation
    • microglia activation accompanies motor neuron degeneration   (MGI Ref ID J:181019)
View Research Applications

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

Endocrine Deficiency Research

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 OriginC57BL/Fa
Gene Symbol and Name Vps54, vacuolar protein sorting 54 (yeast)
Chromosome 11
Gene Common Name(s) 5330404P15Rik; HCC8; PPP1R164; 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]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

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 related

Ait-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]

Broch-Lips M; Pedersen TH; Riisager A; Schmitt-John T; Nielsen OB. 2013. Neuro-muscular function in the wobbler murine model of primary motor neuronopathy. Exp Neurol 248:406-15. [PubMed: 23872513]  [MGI Ref ID J:203704]

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]

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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]

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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]

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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]

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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]

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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]

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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]

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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 & HusbandryWhen 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


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

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2140.00
Animals Provided

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 10 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).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2782.00
Animals Provided

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 10 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).

View USA Canada and Mexico Pricing View International Pricing

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

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Payment Terms and Conditions

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


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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JAX® Mice
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JAX® Services
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Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
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Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries regarding Terms of Use

Contracts Administration

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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