Strain Name:

B6SJL-Tg(SOD1*G93A)1Gur/J

Stock Number:

002726

Availability:

Level 3

Use Restrictions Apply, see Terms of Use

Description

Strain Information

Former Names B6SJL-Tg(SOD1-G93A)1Gur/J    (Changed: 11-JUL-07 )
Type Mutant Strain; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Mating SystemF1 x Hemizygote         (Female x Male)
Specieslaboratory mouse
GenerationN?+43 (20-DEC-06)
 
Donating Investigator Mark Gurney,   deCode Genetics

Appearance
multiple coat colors
Related Genotype: segregating for a, A, Oca2p, Tyrc and Pde6brd1

Description
Mice hemizygous for this SOD1-G93A (also called G93A-SOD1) transgene are viable and fertile, with transgenic expression of a G93A mutant form of human SOD1. This founder line (often referred to as G1H) is reported to have high transgene copy number. Hemizygotes exhibit a phenotype similar to amyotrophic lateral sclerosis (ALS) in humans; becoming paralyzed in one or more limbs with paralysis due to loss of motor neurons from the spinal cord. Transgenic mice have a life span of approximately 19-23 weeks. These SOD1-G93A (also called G93A-SOD1) transgenic mice may be useful in studying neuromuscular disorders, including Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease).

Development
The SOD1-G93A (or G93A-SOD1) transgene was designed with a mutant human SOD1 gene (harboring a single amino acid substitution of glycine to alanine at codon 93) driven by its endogenous human SOD1 promoter. This transgene was injected into fertilized B6SJLF1 mouse eggs and founder animals were obtained. Transgenic mice on a mixed B6SJL genetic background were sent to The Jackson Laboratory.

Control Information

  Control
   Noncarrier
   002297 B6SJL-Tg(SOD1)2Gur/J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Tg(SOD1*G93A)1Gur allele
004435   B6.Cg-Tg(SOD1*G93A)1Gur/J
View Strains carrying   Tg(SOD1*G93A)1Gur     (1 strain)

View Strains carrying other alleles of SOD1     (9 strains)

Additional Web Information

Genetic Quality Control Annual Report
JAX® NOTES, Winter 2008; 512. Explaining the human calcium and sodium palates.
Progress on 'Lou Gehrig's Disease', Science Magazine.

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

View Mammalian Phenotype Terms

Mammalian Phenotype Terms
      assigned by genotype

Tg(SOD1*G93A)1Gur/0

        involves: C57BL/6 * SJL
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:130581)
    • increased survival on C57BL/6J background (50% survival at 157.1+/-9.3 days) in contrast to B6SJL background (50% survival at 128.9+/-9.1 days)
  • nervous system phenotype
  • abnormal neuron physiology (MGI Ref ID J:130581)
    • increased expression of unfolded protein response target genes by 50 days of age in motor neurons
  • abnormal spinal cord morphology (MGI Ref ID J:76718)
    • exhibit neurofilament-rich spheroids at 82-days of age, similar to those seen in human amyotrophic lateral sclerosis; other neuronal intermediate filament proteins (alpha-internexin, peripherin) also accumulate in the spheroids
    • more spheroids are seen in cervical and thoracic regions compared to lumbar and sacral spinal cord in early symptomatic mutants, however similar numbers at all spinal cord levels are seen in older mutants
    • spheroids are more frequently found in the anterior horn and in the anterior and lateral columns of the white matter than in the posterior horn
    • exhibit thickened dystrophic neurites filled with immunoreactive neurofilament-rich inclusions
    • motor neuron degeneration (MGI Ref ID J:76718)
      • develop motor neuron disease; exhibit degenerating motor neurons filled with perikaryal vacuoles in the anterior and lateral horns
      • significant decrease in sciatic motor neuron survival by 90 days of age
  • gliosis (MGI Ref ID J:130581)
    • gliosis and microglial activation are seen in the spinal cord by 90 days of age
    • astrocytosis (MGI Ref ID J:76718)
      • exhibit astrocytosis in spinal cord, mainly in the anterior and lateral horns
  • behavior/neurological phenotype
  • abnormal motor capabilities/coordination/movement (MGI Ref ID J:76718)
    • the first signs of motor neuron disease, hyperflexia, crossed spread of spinal reflexes, and shaking of the limbs when suspended in the air, occur by 91 days of age
    • abnormal gait (MGI Ref ID J:130581)
      • impairment in walking patterns with reduced stride length beginning at 90 days of age
    • abnormal grip strength (MGI Ref ID J:130581)
      • steady decline in paw grip endurance beginning at 77 days of age
    • paralysis (MGI Ref ID J:76718)
      • end-stage disease occurs at an average of 136 days, with mutants exhibiting severe paralysis and inability to forge for food or water
      • hindlimb paralysis (MGI Ref ID J:76718)
        • develop a progressive worsening paresis involving primarily the hind limbs with atrophy of the skeletal musculature
  • muscle phenotype
  • abnormal skeletal muscle morphology (MGI Ref ID J:76718)
    • develop atrophy of skeletal musculature
  • adipose tissue phenotype
  • abnormal adipose tissue morphology (MGI Ref ID J:91800)
    • exhibit reduced adipose tissue accumulation
    • abnormal fat pad morphology (MGI Ref ID J:91800)
      • epididymal and retroperitoneal white adipose tissue is markedly reduced or almost nonexistent in the asymptomatic phase of the disease
  • growth/size phenotype
  • decreased body weight (MGI Ref ID J:91800)
    • lower body mass
    • weight loss (MGI Ref ID J:130581)
      • body weight begins to significantly decrease at 77 days of age
  • homeostasis/metabolism phenotype
  • abnormal circulating hormone level (MGI Ref ID J:91800)
    • decreased circulating insulin level (MGI Ref ID J:91800)
    • decreased circulating leptin level (MGI Ref ID J:91800)
      • plasma leptin levels are diminished
    • increased circulating corticosterone level (MGI Ref ID J:91800)
  • abnormal energy expenditure (MGI Ref ID J:91800)
    • exhibit increased energy expenditure at rest
  • increased oxygen consumption (MGI Ref ID J:91800)
    • exhibit higher rates of total oxygen consumption

Tg(SOD1*G93A)1Gur/?

        involves: C57BL/6 * SJL
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:107901)
    • mice reach end stage by day 147 +/-2.5 SEM (standard error of measurement)
  • nervous system phenotype
  • abnormal spinal cord morphology (MGI Ref ID J:107901)
    • degeneration of the motor neurons in the cervical and lumbar spinal cord is visible at end stage with astrocytosis and vacuolization
    • decreased motor neuron number (MGI Ref ID J:107901)
      • 50% of motor neurons in the cervical spinal cord and 60% in lumbar spinal cord are degenerated at day 152
  • astrocytosis (MGI Ref ID J:107901)
    • astrocytosis is evident in the spinal cord
  • behavior/neurological phenotype
  • hypoactivity (MGI Ref ID J:107901)
    • mice exhibit decreased locomotor activity compared to wild-type mice after day 115
  • growth/size phenotype
  • decreased body weight (MGI Ref ID J:107901)
    • body weights are lower than in wild-type mice and continues to deteriorate after day 115

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

Tg(SOD1*G93A)1Gur/0

        B6.Cg-Tg(SOD1*G93A)1Gur/J
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:115355)
    • mean time from onset of tremors to death is 16.5 +/-9.3 days
    • increased survival on C57BL/6J background (50% survival at 157.1+/-9.3 days) in contrast to B6SJL background (50% survival at 128.9+/-9.1 days)
  • behavior/neurological phenotype
  • abnormal gait (MGI Ref ID J:115355)
    • exhibited longer stride and stance times as compared to C57BL/6J controls, however swing time difference was not significant
  • tremors (MGI Ref ID J:115355)
    • observed hindlimb tremors when suspended by tail at 142.3 +/- 10.6 days (approx. 20 weeks)
View Research Applications

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

Metabolism Research

Mouse/Human Gene Homologs
amyotrophic lateral sclerosis (ALS)

Neurobiology Research
Amyotrophic Lateral Sclerosis (ALS)
Metabolic Defects
Neurodegeneration

Genes & Alleles

Gene & Allele Information

 
Allele Symbol Tg(SOD1*G93A)1Gur
Allele Name transgene insertion 1, Mark E Gurney
Allele Type Transgenic (random, expressed)
Common Name(s) (G93A)Tg+; G1H; G93A; G93A SOD1; G93A+; G93A-SOD1; SOD1 G93A; SOD1 Tg; SOD1G93A; Tg(G93A-SOD1)1Gur; Tg(SOD1-G93A)1Gur; TgN(SOD1-G93A)1Gur; TgN[SOD1-G93A]1Gur; hSOD1G93A;
Mutation Made By Mark Gurney,   deCode Genetics
Strain of Origin(C57BL/6 x SJL)F1
Expressed Gene SOD1, superoxide dismutase 1, soluble, human
Promoter SOD1, superoxide dismutase 1, soluble, human
General Note This line, G1H, was derived from the original G1 line (now designated Tg(SOD1*G93A)2Gur) reported in J:32665.

Transgenic mice on a background that involves C57BL/6 and SJL express high levels of the transgene with a 4-fold increase in SOD activity, and exhibit a phenotype similar to amyotrophic lateral sclerosis (ALS) in humans. Hemizygous transgenic mice become paralyzed in one or more limbs and have a life span of approximately 19-23 weeks. Paralysis is due to loss of motor neurons from the spinal cord.

Molecular Note This transgenic subline (designated G1H in J:76718) is derived from the G1 parental transgenic line (originally described in J:32665). This line carries a 40% expansion in transgene copy number compared to the original G1 line (described in J:32665, in MGI as Tg(SOD1*G93A)2Gur). The transgene construct is composed of the human SOD1 gene carrying a glycine to alanine transition at position 93 (G93A). The G93A mutation does not alter the activity of the protein. This line carries a high copy number. [MGI Ref ID J:32665] [MGI Ref ID J:76718]
 

Genotyping

Genotyping Information

Genotyping Protocols

Tg(SOD1*G93A)1Gur, QPCR, vers. 2
Tg(SOD), MCA, vers. 2
Tg(SOD1), STD PCR, vers. 1

Helpful Links

Optimizing PCR Protocols

References

References

Selected Reference(s)

Gurney ME; Pu H; Chiu AY; Dal Canto MC; Polchow CY; Alexander DD; Caliendo J; Hentati A; Kwon YW; Deng HX; Chen W; Zhai P; Sufit RL; Siddique T. 1994. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation [see comments] [published erratum appears in Science 1995 Jul 14;269(5221):149] Science 264(5166):1772-5. [PubMed: 8209258]  [MGI Ref ID J:32665]

Additional References

Andreassen OA; Jenkins BG; Dedeoglu A; Ferrante KL; Bogdanov MB; Kaddurah-Daouk R; Beal MF. 2001. Increases in cortical glutamate concentrations in transgenic amyotrophic lateral sclerosis mice are attenuated by creatine supplementation. J Neurochem 77(2):383-90. [PubMed: 11299300]  [MGI Ref ID J:68835]

Azari MF; Galle A; Lopes EC; Kurek J; Cheema SS. 2001. Leukemia inhibitory factor by systemic administration rescues spinal motor neurons in the SOD1 G93A murine model of familial amyotrophic lateral sclerosis. Brain Res 922(1):144-7. [PubMed: 11730713]  [MGI Ref ID J:73366]

Chiu AY; Zhai P; Dal Canto MC; Peters TM; Kwon YW; Prattis SM; Gurney ME. 1995. Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis. Mol Cell Neurosci 6(4):349-62. [PubMed: 8846004]  [MGI Ref ID J:80625]

Cifuentes-Diaz C; Nicole S; Velasco ME; Borra-Cebrian C; Panozzo C; Frugier T; Millet G; Roblot N; Joshi V; Melki J. 2002. Neurofilament accumulation at the motor endplate and lack of axonal sprouting in a spinal muscular atrophy mouse model. Hum Mol Genet 11(12):1439-47. [PubMed: 12023986]  [MGI Ref ID J:76936]

Dupuis L; Oudart H; Rene F; Gonzalez de Aguilar JL; Loeffler JP. 2004. Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model. Proc Natl Acad Sci U S A 101(30):11159-64. [PubMed: 15263088]  [MGI Ref ID J:91800]

Guegan C; Vila M; Rosoklija G; Hays AP; Przedborski S. 2001. Recruitment of the mitochondrial-dependent apoptotic pathway in amyotrophic lateral sclerosis. J Neurosci 21(17):6569-76. [PubMed: 11517246]  [MGI Ref ID J:71177]

Guo H; Lai L; Butchbach ME; Stockinger MP; Shan X; Bishop GA; Lin CL. 2003. Increased expression of the glial glutamate transporter EAAT2 modulates excitotoxicity and delays the onset but not the outcome of ALS in mice. Hum Mol Genet 12(19):2519-32. [PubMed: 12915461]  [MGI Ref ID J:85996]

Hamson DK; Hu JH; Krieger C; Watson NV. 2002. Lumbar motoneuron fate in a mouse model of amyotrophic lateral sclerosis. Neuroreport 13(17):2291-4. [PubMed: 12488813]  [MGI Ref ID J:89674]

Kabashi E; Agar JN; Taylor DM; Minotti S; Durham HD. 2004. Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis. J Neurochem 89(6):1325-35. [PubMed: 15189335]  [MGI Ref ID J:92215]

Kang SJ; Sanchez I; Jing N; Yuan J. 2003. Dissociation between neurodegeneration and caspase-11-mediated activation of caspase-1 and caspase-3 in a mouse model of amyotrophic lateral sclerosis. J Neurosci 23(13):5455-60. [PubMed: 12843244]  [MGI Ref ID J:84374]

Kilic E; Weishaupt JH; Kilic U; Rohde G; Yulug B; Peters K; Hermann DM; Bahr M. 2004. The superoxide dismutase1 (sod1) G93A mutation does not promote neuronal injury after focal brain ischemia and optic nerve transection in mice. Neuroscience 128(2):359-64. [PubMed: 15350647]  [MGI Ref ID J:92468]

Li PA; He Q; Cao T; Yong G; Szauter KM; Fong KS; Karlsson J; Keep MF; Csiszar K. 2004. Up-regulation and altered distribution of lysyl oxidase in the central nervous system of mutant SOD1 transgenic mouse model of amyotrophic lateral sclerosis. Brain Res Mol Brain Res 120(2):115-22. [PubMed: 14741400]  [MGI Ref ID J:88033]

Lino MM; Schneider C; Caroni P. 2002. Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron disease. J Neurosci 22(12):4825-32. [PubMed: 12077179]  [MGI Ref ID J:77533]

Murakami T; Ilieva H; Shiote M; Nagata T; Nagano I; Shoji M; Abe K. 2003. Hypoxic induction of vascular endothelial growth factor is selectively impaired in mice carrying the mutant SOD1 gene. Brain Res 989(2):231-7. [PubMed: 14556945]  [MGI Ref ID J:86204]

Pedersen WA; Luo H; Kruman I; Kasarskis E; Mattson MP. 2000. The prostate apoptosis response-4 protein participates in motor neuron degeneration in amyotrophic lateral sclerosis. FASEB J 14(7):913-24. [PubMed: 10783145]  [MGI Ref ID J:61806]

Tu PH; Raju P; Robinson KA; Gurney ME; Trojanowski JQ; Lee VM. 1996. Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions. Proc Natl Acad Sci U S A 93(7):3155-60. [PubMed: 8610185]  [MGI Ref ID J:76718]

West M; Mhatre M; Ceballos A; Floyd RA; Grammas P; Gabbita SP; Hamdheydari L; Mai T; Mou S; Pye QN; Stewart C; West S; Williamson KS; Zemlan F; Hensley K. 2004. The arachidonic acid 5-lipoxygenase inhibitor nordihydroguaiaretic acid inhibits tumor necrosis factor alpha activation of microglia and extends survival of G93A-SOD1 transgenic mice. J Neurochem 91(1):133-43. [PubMed: 15379894]  [MGI Ref ID J:93284]

Wootz H; Hansson I; Korhonen L; Napankangas U; Lindholm D. 2004. Caspase-12 cleavage and increased oxidative stress during motoneuron degeneration in transgenic mouse model of ALS. Biochem Biophys Res Commun 322(1):281-6. [PubMed: 15313203]  [MGI Ref ID J:92029]

Wu AS; Kiaei M; Aguirre N; Crow JP; Calingasan NY; Browne SE; Beal MF. 2003. Iron porphyrin treatment extends survival in a transgenic animal model of amyotrophic lateral sclerosis. J Neurochem 85(1):142-50. [PubMed: 12641736]  [MGI Ref ID J:82680]

Yoshihara T; Ishigaki S; Yamamoto M; Liang Y; Niwa J; Takeuchi H; Doyu M; Sobue G. 2002. Differential expression of inflammation- and apoptosis-related genes in spinal cords of a mutant SOD1 transgenic mouse model of familial amyotrophic lateral sclerosis. J Neurochem 80(1):158-67. [PubMed: 11796754]  [MGI Ref ID J:78835]

Tg(SOD1*G93A)1Gur related

Achilli F; Boyle S; Kieran D; Chia R; Hafezparast M; Martin JE; Schiavo G; Greensmith L; Bickmore W; Fisher EM. 2005. The SOD1 transgene in the G93A mouse model of amyotrophic lateral sclerosis lies on distal mouse chromosome 12. Amyotroph Lateral Scler Other Motor Neuron Disord 6(2):111-4. [PubMed: 16036435]  [MGI Ref ID J:100363]

Alegre ML; Shiels H; Thompson CB; Gajewski TF. 1998. Expression and function of CTLA-4 in Th1 and Th2 cells. J Immunol 161(7):3347-56. [PubMed: 9759851]  [MGI Ref ID J:115196]

Alexander GM; Deitch JS; Seeburger JL; Del Valle L; Heiman-Patterson TD. 2000. Elevated cortical extracellular fluid glutamate in transgenic mice expressing human mutant (G93A) Cu/Zn superoxide dismutase. J Neurochem 74(4):1666-73. [PubMed: 10737625]  [MGI Ref ID J:61168]

Alexander GM; Erwin KL; Byers N; Deitch JS; Augelli BJ; Blankenhorn EP; Heiman-Patterson TD. 2004. Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse model of ALS. Brain Res Mol Brain Res 130(1-2):7-15. [PubMed: 15519671]  [MGI Ref ID J:94198]

Almer G; Vukosavic S; Romero N; Przedborski S. 1999. Inducible nitric oxide synthase up-regulation in a transgenic mouse model of familial amyotrophic lateral sclerosis. J Neurochem 72(6):2415-25. [PubMed: 10349851]  [MGI Ref ID J:55026]

Ammassari-Teule M; Restivo L; Pietteur V; Passino E. 2001. Learning about the context in genetically-defined mice. Behav Brain Res 125(1-2):195-204. [PubMed: 11682111]  [MGI Ref ID J:92773]

Andreassen OA; Ferrante RJ; Klivenyi P; Klein AM; Dedeoglu A; Albers DS; Kowall NW; Beal MF. 2001. Transgenic ALS mice show increased vulnerability to the mitochondrial toxins MPTP and 3-nitropropionic acid. Exp Neurol 168(2):356-63. [PubMed: 11259123]  [MGI Ref ID J:127805]

Andreassen OA; Ferrante RJ; Klivenyi P; Klein AM; Shinobu LA; Epstein CJ; Beal MF. 2000. Partial deficiency of manganese superoxide dismutase exacerbates a transgenic mouse model of amyotrophic lateral sclerosis. Ann Neurol 47(4):447-55. [PubMed: 10762155]  [MGI Ref ID J:62381]

Atkin JD; Farg MA; Turner BJ; Tomas D; Lysaght JA; Nunan J; Rembach A; Nagley P; Beart PM; Cheema SS; Horne MK. 2006. Induction of the unfolded protein response in familial amyotrophic lateral sclerosis and association of protein-disulfide isomerase with superoxide dismutase 1. J Biol Chem 281(40):30152-65. [PubMed: 16847061]  [MGI Ref ID J:117296]

Atkin JD; Scott RL; West JM; Lopes E; Quah AK; Cheema SS. 2005. Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis. Neuromuscul Disord 15(5):377-88. [PubMed: 15833433]  [MGI Ref ID J:132546]

Avossa D; Grandolfo M; Mazzarol F; Zatta M; Ballerini L. 2006. Early signs of motoneuron vulnerability in a disease model system: Characterization of transverse slice cultures of spinal cord isolated from embryonic ALS mice. Neuroscience 138(4):1179-94. [PubMed: 16442737]  [MGI Ref ID J:106998]

Azzouz M; Krezel W; Dolle P; Vodouhe C; Warter JM; Poindron P; Borg J. 1999. Compensatory mechanism of motor defect in SOD1 transgenic mice by overactivation of striatal cholinergic neurons. Neuroreport 10(5):1013-8. [PubMed: 10321477]  [MGI Ref ID J:56222]

Banerjee R; Mosley RL; Reynolds AD; Dhar A; Jackson-Lewis V; Gordon PH; Przedborski S; Gendelman HE. 2008. Adaptive immune neuroprotection in G93A-SOD1 amyotrophic lateral sclerosis mice. PLoS ONE 3(7):e2740. [PubMed: 18648532]  [MGI Ref ID J:138237]

Barneoud P; Curet O. 1999. Beneficial effects of lysine acetylsalicylate, a soluble salt of aspirin, on motor performance in a transgenic model of amyotrophic lateral sclerosis. Exp Neurol 155(2):243-51. [PubMed: 10072299]  [MGI Ref ID J:53856]

Basso M; Massignan T; Samengo G; Cheroni C; De Biasi S; Salmona M; Bendotti C; Bonetto V. 2006. Insoluble mutant SOD1 is partly oligoubiquitinated in amyotrophic lateral sclerosis mice. J Biol Chem 281(44):33325-35. [PubMed: 16943203]  [MGI Ref ID J:117191]

Beers DR; Henkel JS; Xiao Q; Zhao W; Wang J; Yen AA; Siklos L; McKercher SR; Appel SH. 2006. Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A 103(43):16021-6. [PubMed: 17043238]  [MGI Ref ID J:116087]

Bilsland LG; Dick JR; Pryce G; Petrosino S; Di Marzo V; Baker D; Greensmith L. 2006. Increasing cannabinoid levels by pharmacological and genetic manipulation delay disease progression in SOD1 mice. FASEB J 20(7):1003-5. [PubMed: 16571781]  [MGI Ref ID J:111498]

Bilsland LG; Nirmalananthan N; Yip J; Greensmith L; Duchen MR. 2008. Expression of mutant SOD1 in astrocytes induces functional deficits in motoneuron mitochondria. J Neurochem 107(5):1271-83. [PubMed: 18808448]  [MGI Ref ID J:142837]

Boston-Howes W; Gibb SL; Williams EO; Pasinelli P; Brown RH Jr; Trotti D. 2006. Caspase-3 cleaves and inactivates the glutamate transporter EAAT2. J Biol Chem 281(20):14076-84. [PubMed: 16567804]  [MGI Ref ID J:113480]

Browne SE; Yang L; DiMauro JP; Fuller SW; Licata SC; Beal MF. 2006. Bioenergetic abnormalities in discrete cerebral motor pathways presage spinal cord pathology in the G93A SOD1 mouse model of ALS. Neurobiol Dis 22(3):599-610. [PubMed: 16616851]  [MGI Ref ID J:111280]

Canton T; Pratt J; Stutzmann JM; Imperato A; Boireau A. 1998. Glutamate uptake is decreased tardively in the spinal cord of FALS mice. Neuroreport 9(5):775-8. [PubMed: 9579663]  [MGI Ref ID J:103582]

Casoni F; Basso M; Massignan T; Gianazza E; Cheroni C; Salmona M; Bendotti C; Bonetto V. 2005. Protein nitration in a mouse model of familial amyotrophic lateral sclerosis: possible multifunctional role in the pathogenesis. J Biol Chem 280(16):16295-304. [PubMed: 15699043]  [MGI Ref ID J:98724]

Chandra S; Gallardo G; Fernandez-Chacon R; Schluter OM; Sudhof TC. 2005. Alpha-synuclein cooperates with CSPalpha in preventing neurodegeneration. Cell 123(3):383-96. [PubMed: 16269331]  [MGI Ref ID J:115193]

Chang Y; Kong Q; Shan X; Tian G; Ilieva H; Cleveland DW; Rothstein JD; Borchelt DR; Wong PC; Lin CL. 2008. Messenger RNA oxidation occurs early in disease pathogenesis and promotes motor neuron degeneration in ALS. PLoS ONE 3(8):e2849. [PubMed: 18682740]  [MGI Ref ID J:140123]

Chen XJ; Levedakou EN; Millen KJ; Wollmann RL; Soliven B; Popko B. 2007. Proprioceptive sensory neuropathy in mice with a mutation in the cytoplasmic Dynein heavy chain 1 gene. J Neurosci 27(52):14515-24. [PubMed: 18160659]  [MGI Ref ID J:131126]

Chiu AY; Zhai P; Dal Canto MC; Peters TM; Kwon YW; Prattis SM; Gurney ME. 1995. Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis. Mol Cell Neurosci 6(4):349-62. [PubMed: 8846004]  [MGI Ref ID J:80625]

Cho KJ; Chung YH; Shin C; Shin DH; Kim YS; Gurney ME; Lee KW; Cha CI. 1999. Reactive astrocytes express p53 in the spinal cord of transgenic mice expressing a human Cu/Zn SOD mutation Neuroreport 10(18):3939-43. [PubMed: 10716237]  [MGI Ref ID J:61275]

Chung YH; Joo KM; Lee YJ; Lee WB; Lee KH; Cha CI. 2004. Enhanced expression of erythropoietin in the central nervous system of SOD1(G93A) transgenic mice. Brain Res 1016(2):272-80. [PubMed: 15246865]  [MGI Ref ID J:91260]

Chung YH; Joo KM; Lim HC; Cho MH; Kim D; Lee WB; Cha CI. 2005. Immunohistochemical study on the distribution of phosphorylated extracellular signal-regulated kinase (ERK) in the central nervous system of SOD1(G93A) transgenic mice. Brain Res 1050(1-2):203-9. [PubMed: 15978558]  [MGI Ref ID J:99567]

Chung YH; Joo KM; Nam RH; Cho MH; Kim DJ; Lee WB; Cha CI. 2005. Decreased expression of calretinin in the cerebral cortex and hippocampus of SOD1G93A transgenic mice. Brain Res 1035(1):105-9. [PubMed: 15713283]  [MGI Ref ID J:97425]

Copray JC; Jaarsma D; Kust BM; Bruggeman RW; Mantingh I; Brouwer N; Boddeke HW. 2003. Expression of the low affinity neurotrophin receptor p75 in spinal motoneurons in a transgenic mouse model for amyotrophic lateral sclerosis. Neuroscience 116(3):685-94. [PubMed: 12573711]  [MGI Ref ID J:132258]

Crosio C; Casciati A; Iaccarino C; Rotilio G; Carri MT. 2006. Bcl2a1 serves as a switch in death of motor neurons in amyotrophic lateral sclerosis. Cell Death Differ 13(12):2150-3. [PubMed: 16710367]  [MGI Ref ID J:132252]

Damiano M; Starkov AA; Petri S; Kipiani K; Kiaei M; Mattiazzi M; Flint Beal M; Manfredi G. 2006. Neural mitochondrial Ca capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-superoxide dismutase mutant mice. J Neurochem 96(5):1349-61. [PubMed: 16478527]  [MGI Ref ID J:106152]

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Pehar M; Cassina P; Vargas MR; Xie Y; Beckman JS; Massa SM; Longo FM; Barbeito L. 2006. Modulation of p75-dependent motor neuron death by a small non-peptidyl mimetic of the neurotrophin loop 1 domain. Eur J Neurosci 24(6):1575-80. [PubMed: 17004921]  [MGI Ref ID J:112914]

Pena-Altamira E; Crochemore C; Virgili M; Contestabile A. 2005. Neurochemical correlates of differential neuroprotection by long-term dietary creatine supplementation. Brain Res 1058(1-2):183-8. [PubMed: 16140286]  [MGI Ref ID J:101935]

Perrin FE; Boisset G; Docquier M; Schaad O; Descombes P; Kato AC. 2005. No widespread induction of cell death genes occurs in pure motoneurons in an amyotrophic lateral sclerosis mouse model. Hum Mol Genet 14(21):3309-20. [PubMed: 16192287]  [MGI Ref ID J:102747]

Petersen A; Hansson O; Puschban Z; Sapp E; Romero N; Castilho RF; Sulzer D; Rice M; DiFiglia M; Przedborski S; Brundin P. 2001. Mice transgenic for exon 1 of the Huntington's disease gene display reduced striatal sensitivity to neurotoxicity induced by dopamine and 6-hydroxydopamine. Eur J Neurosci 14(9):1425-35. [PubMed: 11722604]  [MGI Ref ID J:128172]

Petri S; Kiaei M; Damiano M; Hiller A; Wille E; Manfredi G; Calingasan NY; Szeto HH; Beal MF. 2006. Cell-permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis. J Neurochem 98(4):1141-8. [PubMed: 16895581]  [MGI Ref ID J:119276]

Petri S; Kiaei M; Wille E; Calingasan NY; Flint Beal M. 2006. Loss of Fas ligand-function improves survival in G93A-transgenic ALS mice. J Neurol Sci 251(1-2):44-9. [PubMed: 17049562]  [MGI Ref ID J:129276]

Rakhit R; Robertson J; Vande Velde C; Horne P; Ruth DM; Griffin J; Cleveland DW; Cashman NR; Chakrabartty A. 2007. An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS. Nat Med 13(6):754-9. [PubMed: 17486090]  [MGI Ref ID J:133800]

Reinholz MM; Merkle CM; Poduslo JF. 1999. Therapeutic benefits of putrescine-modified catalase in a transgenic mouse model of familial amyotrophic lateral sclerosis. Exp Neurol 159(1):204-16. [PubMed: 10486188]  [MGI Ref ID J:57736]

Saito Y; Yokota T; Mitani T; Ito K; Anzai M; Miyagishi M; Taira K; Mizusawa H. 2005. Transgenic small interfering RNA halts amyotrophic lateral sclerosis in a mouse model. J Biol Chem 280(52):42826-30. [PubMed: 16221675]  [MGI Ref ID J:105911]

Schoenebeck B; Bader V; Zhu XR; Schmitz B; Lubbert H; Stichel CC. 2005. Sgk1, a cell survival response in neurodegenerative diseases. Mol Cell Neurosci 30(2):249-64. [PubMed: 16125969]  [MGI Ref ID J:129832]

Schonhoff CM; Matsuoka M; Tummala H; Johnson MA; Estevez AG; Wu R; Kamaid A; Ricart KC; Hashimoto Y; Gaston B; Macdonald TL; Xu Z; Mannick JB. 2006. S-nitrosothiol depletion in amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A 103(7):2404-9. [PubMed: 16461917]  [MGI Ref ID J:106061]

Schutz B. 2005. Imbalanced excitatory to inhibitory synaptic input precedes motor neuron degeneration in an animal model of amyotrophic lateral sclerosis. Neurobiol Dis 20(1):131-40. [PubMed: 16137574]  [MGI Ref ID J:102615]

Shan X; Hu JH; Cayabyab FS; Krieger C. 2005. Increased phospho-adducin immunoreactivity in a murine model of amyotrophic lateral sclerosis. Neuroscience 134(3):833-46. [PubMed: 15994023]  [MGI Ref ID J:104428]

Sharp PS; Akbar MT; Bouri S; Senda A; Joshi K; Chen HJ; Latchman DS; Wells DJ; de Belleroche J. 2008. Protective effects of heat shock protein 27 in a model of ALS occur in the early stages of disease progression. Neurobiol Dis 30(1):42-55. [PubMed: 18255302]  [MGI Ref ID J:141624]

Sharp PS; Dick JR; Greensmith L. 2005. The effect of peripheral nerve injury on disease progression in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis. Neuroscience 130(4):897-910. [PubMed: 15652988]  [MGI Ref ID J:132280]

Smittkamp SE; Brown JW; Stanford JA. 2008. Time-course and characterization of orolingual motor deficits in B6SJL-Tg(SOD1-G93A)1Gur/J mice. Neuroscience 151(2):613-21. [PubMed: 18061359]  [MGI Ref ID J:130811]

Spalloni A; Geracitano R; Berretta N; Sgobio C; Bernardi G; Mercuri NB; Longone P; Ammassari-Teule M. 2006. Molecular and synaptic changes in the hippocampus underlying superior spatial abilities in pre-symptomatic G93A+/+ mice overexpressing the human Cu/Zn superoxide dismutase (Gly(93) --> ALA) mutation. Exp Neurol 197(2):505-14. [PubMed: 16309674]  [MGI Ref ID J:104511]

Spooren WP; Hengerer B. 2000. DNA laddering and caspase 3-like activity in the spinal cord of a mouse model of familial amyotrophic lateral sclerosis Cell Mol Biol (Noisy-Le-Grand) 46(1):63-9. [PubMed: 10726972]  [MGI Ref ID J:61246]

Stam NC; Nithianantharajah J; Howard ML; Atkin JD; Cheema SS; Hannan AJ. 2008. Sex-specific behavioural effects of environmental enrichment in a transgenic mouse model of amyotrophic lateral sclerosis. Eur J Neurosci 28(4):717-23. [PubMed: 18702691]  [MGI Ref ID J:140578]

Stieber A; Gonatas JO; Collard J; Meier J; Julien J; Schweitzer P; Gonatas NK. 2000. The neuronal Golgi apparatus is fragmented in transgenic mice expressing a mutant human SOD1, but not in mice expressing the human NF-H gene. J Neurol Sci 173(1):63-72. [PubMed: 10675581]  [MGI Ref ID J:127959]

Stieber A; Gonatas JO; Gonatas NK. 2000. Aggregates of mutant protein appear progressively in dendrites, in periaxonal processes of oligodendrocytes, and in neuronal and astrocytic perikarya of mice expressing the SOD1(G93A) mutation of familial amyotrophic lateral sclerosis. J Neurol Sci 177(2):114-23. [PubMed: 10980307]  [MGI Ref ID J:130542]

Takahashi S; Kulkarni AB. 2004. Mutant superoxide dismutase 1 causes motor neuron degeneration independent of cyclin-dependent kinase 5 activation by p35 or p25. J Neurochem 88(5):1295-304. [PubMed: 15009685]  [MGI Ref ID J:88474]

Taylor DM; Gibbs BF; Kabashi E; Minotti S; Durham HD; Agar JN. 2007. Tryptophan 32 potentiates aggregation and cytotoxicity of a copper/zinc superoxide dismutase mutant associated with familial amyotrophic lateral sclerosis. J Biol Chem 282(22):16329-35. [PubMed: 17389599]  [MGI Ref ID J:122717]

Teuchert M; Fischer D; Schwalenstoecker B; Habisch HJ; Bockers TM; Ludolph AC. 2006. A dynein mutation attenuates motor neuron degeneration in SOD1(G93A) mice. Exp Neurol 198(1):271-4. [PubMed: 16427626]  [MGI Ref ID J:107901]

Tovar-y-Romo LB; Tapia R. 2006. Cerebral neurons of transgenic ALS mice are vulnerable to glutamate release stimulation but not to increased extracellular glutamate due to transport blockade. Exp Neurol 199(2):281-90. [PubMed: 16364298]  [MGI Ref ID J:111208]

Trieu VN; Liu R; Liu XP; Uckun FM. 2000. A specific inhibitor of janus kinase-3 increases survival in a transgenic mouse model of amyotrophic lateral sclerosis. Biochem Biophys Res Commun 267(1):22-5. [PubMed: 10623568]  [MGI Ref ID J:59400]

Tu PH; Raju P; Robinson KA; Gurney ME; Trojanowski JQ; Lee VM. 1996. Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions. Proc Natl Acad Sci U S A 93(7):3155-60. [PubMed: 8610185]  [MGI Ref ID J:76718]

Turner BJ; Cheah IK; Macfarlane KJ; Lopes EC; Petratos S; Langford SJ; Cheema SS. 2003. Antisense peptide nucleic acid-mediated knockdown of the p75 neurotrophin receptor delays motor neuron disease in mutant SOD1 transgenic mice. J Neurochem 87(3):752-63. [PubMed: 14535957]  [MGI Ref ID J:86152]

Urushitani M; Sik A; Sakurai T; Nukina N; Takahashi R; Julien JP. 2006. Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat Neurosci 9(1):108-18. [PubMed: 16369483]  [MGI Ref ID J:105360]

Van Damme P; Braeken D; Callewaert G; Robberecht W; Van Den Bosch L. 2005. GluR2 deficiency accelerates motor neuron degeneration in a mouse model of amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 64(7):605-12. [PubMed: 16042312]  [MGI Ref ID J:104951]

Veldink JH; Bar PR; Joosten EA; Otten M; Wokke JH; van den Berg LH. 2003. Sexual differences in onset of disease and response to exercise in a transgenic model of ALS. Neuromuscul Disord 13(9):737-43. [PubMed: 14561497]  [MGI Ref ID J:128781]

Vijayvergiya C; Beal MF; Buck J; Manfredi G. 2005. Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice. J Neurosci 25(10):2463-70. [PubMed: 15758154]  [MGI Ref ID J:134416]

Virgili M; Crochemore C; Pena-Altamira E; Contestabile A. 2006. Regional and temporal alterations of ODC/polyamine system during ALS-like neurodegenerative motor syndrome in G93A transgenic mice. Neurochem Int 48(3):201-7. [PubMed: 16290266]  [MGI Ref ID J:129220]

Vukosavic S; Dubois-Dauphin M; Romero N; Przedborski S. 1999. Bax and Bcl-2 interaction in a transgenic mouse model of familial amyotrophic lateral sclerosis. J Neurochem 73(6):2460-8. [PubMed: 10582606]  [MGI Ref ID J:58488]

Vukosavic S; Stefanis L; Jackson-Lewis V; Guegan C; Romero N; Chen C; Dubois-Dauphin M; Przedborski S. 2000. Delaying caspase activation by bcl-2: A clue to disease retardation in a transgenic mouse model of amyotrophic lateral sclerosis J Neurosci 20(24):9119-25. [PubMed: 11124989]  [MGI Ref ID J:66731]

Wang J; Slunt H; Gonzales V; Fromholt D; Coonfield M; Copeland NG; Jenkins NA; Borchelt DR. 2003. Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature. Hum Mol Genet 12(21):2753-64. [PubMed: 12966034]  [MGI Ref ID J:86421]

Wang Y; Mao XO; Xie L; Banwait S; Marti HH; Greenberg DA; Jin K. 2007. Vascular endothelial growth factor overexpression delays neurodegeneration and prolongs survival in amyotrophic lateral sclerosis mice. J Neurosci 27(2):304-7. [PubMed: 17215390]  [MGI Ref ID J:117304]

Watanabe S; Nagano S; Duce J; Kiaei M; Li QX; Tucker SM; Tiwari A; Brown RH Jr; Beal MF; Hayward LJ; Culotta VC; Yoshihara S; Sakoda S; Bush AI. 2007. Increased affinity for copper mediated by cysteine 111 in forms of mutant superoxide dismutase 1 linked to amyotrophic lateral sclerosis. Free Radic Biol Med 42(10):1534-42. [PubMed: 17448900]  [MGI Ref ID J:121596]

Wendt W; Lubbert H; Stichel CC. 2008. Upregulation of cathepsin S in the aging and pathological nervous system of mice. Brain Res 1232:7-20. [PubMed: 18694734]  [MGI Ref ID J:140397]

West M; Mhatre M; Ceballos A; Floyd RA; Grammas P; Gabbita SP; Hamdheydari L; Mai T; Mou S; Pye QN; Stewart C; West S; Williamson KS; Zemlan F; Hensley K. 2004. The arachidonic acid 5-lipoxygenase inhibitor nordihydroguaiaretic acid inhibits tumor necrosis factor alpha activation of microglia and extends survival of G93A-SOD1 transgenic mice. J Neurochem 91(1):133-43. [PubMed: 15379894]  [MGI Ref ID J:93284]

Weydt P; Pineda VV; Torrence AE; Libby RT; Satterfield TF; Lazarowski ER; Gilbert ML; Morton GJ; Bammler TK; Strand AD; Cui L; Beyer RP; Easley CN; Smith AC; Krainc D; Luquet S; Sweet IR; Schwartz MW; La Spada AR. 2006. Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration. Cell Metab 4(5):349-62. [PubMed: 17055784]  [MGI Ref ID J:129751]

Wooley CM; Sher RB; Kale A; Frankel WN; Cox GA; Seburn KL. 2005. Gait analysis detects early changes in transgenic SOD1(G93A) mice. Muscle Nerve 32(1):43-50. [PubMed: 15880561]  [MGI Ref ID J:115355]

Wootz H; Hansson I; Korhonen L; Lindholm D. 2006. XIAP decreases caspase-12 cleavage and calpain activity in spinal cord of ALS transgenic mice. Exp Cell Res 312(10):1890-8. [PubMed: 16566922]  [MGI Ref ID J:111367]

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Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX1

Colony Maintenance

Breeding & HusbandryThe strain is maintained by breeding hemizygous carriers (preferably males) to B6SJLF1 hybrids. Expected coat colors from breeding are "White Bellied Agouti, Black, Albino, Tan w/pink eyes."
Mating SystemF1 x Hemizygote         (Female x Male)
Diet Information LabDiet® 5K52/5K67

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Pricing for USA, Canada and Mexico shipping destinations View International pricing
Weeks of AgePrice (US dollars $)GenderGenotypes Provided
5 weeks $197.60Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
6 weeks $201.50Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
7 weeks $205.40Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
8 weeks $209.30Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
9 weeks $213.20Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
10 weeks $217.10Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
Pairs /Price (US dollars $)Pair Genotype
$219.45B6SJLF1/J (100012) x Hemizygous for Tg(SOD1-G93A)1Gur

Additional Supply Details

Supply Notes
  • Shipped at a specific age in weeks. Mice at a precise age in days, littermates and retired breeders are also available.
  • Strains that must be genotyped are not available until five to seven weeks of age.

Pricing for International shipping destinations View USA Canada and Mexico pricing
Weeks of AgePrice (US dollars $)GenderGenotypes Provided
5 weeks $256.90Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
6 weeks $262.00Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
7 weeks $267.10Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
8 weeks $272.20Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
9 weeks $277.30Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
10 weeks $282.40Female or MaleHemizygous for Tg(SOD1-G93A)1Gur
Pairs /Price (US dollars $)Pair Genotype
$285.30B6SJLF1/J (100012) x Hemizygous for Tg(SOD1-G93A)1Gur

Additional Supply Details

Supply Notes
  • Strains that require genotyping are only offered at five weeks of age and older. The time required for sample collection, assay, reporting, and completion of USDA documentation required for international purchases make distribution of younger mice prohibitive. Mice at a precise age in days, littermates and retired breeders are also available. Mice older than 10 weeks of age can be requested by contacting JAX® Mice & Services.

Supply Details

Standard SupplyLevel 3. Up to 50 mice. Larger quantities or custom orders arranged upon request.
Supply Notes

Control Information

  Control
   Noncarrier
   002297 B6SJL-Tg(SOD1)2Gur/J
 
  Considerations for Choosing Controls
  USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
  International - Control Pricing Information for Genetically Engineered Mutant Strains.

General Terms and Conditions


See Terms of Use


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.
Ordering and Purchasing Information

      Purchasing Information
      JAX® Mice Orders
      Surgical Services

Contact Information
Orders & Technical Support
Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


For Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license.

Contact information

General inquiries

Contracts Administration

phone:207-288-6470
fax:207-288-6655

JAX® Mice & Services Conditions of Use

“Each recipient institution, including its employees and other researchers under its control (RECIPIENT), of mice or services using mice from The Jackson Laboratory (TJL) agrees that such mice, descendants of those mice derived by inbreeding or crossbreeding, including unmodified derivatives of those mice or their descendants (“MICE”) shall not be: (i) used for any purpose other than the internal research of the RECIPIENT, (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 with respect to MICE. Acceptance of MICE from TJL shall be deemed agreement by RECIPIENT to these conditions, and departure from these conditions requires The Jackson Laboratory’s prior written authorization.”

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. THE LABORATORY 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, The Jackson Laboratory will, at its option, provide credit or replacement for the MICE or product received or the services provided.

No Liability

In no event shall The Jackson Laboratory, 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 The Jackson Laboratory, its agents or employees. In purchasing or receiving MICE, products or services from The Jackson Laboratory, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges The Jackson Laboratory from all such causes of action or damages, and further agrees to defend and indemnify The Jackson Laboratory from any costs or damages arising out of any third party claims.

MICE and biological materials 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 The Jackson Laboratory’s MICE, pr