Strain Name:

B6.Cg-Tg(SOD1)2Gur/J

Stock Number:

002298

Availability:

Repository-Cryopreserved

Use Restrictions Apply, see Terms of Use

Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Congenic; Mutant Strain; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain B6SJLF2
GenerationN?+10 (30-MAY-04)
 
Donating Investigator IMR Colony,   The Jackson Laboratory

Appearance
black
Related Genotype: a/a

Description
This transgenic strain carries the normal allele of the human SOD1 gene. Originally published as N1029, it expresses the same SOD1 protein level as the transgenic strain carrying the SOD1*G93A transgene (002726), even though the copy number in the SOD1*G93A transgenic is higher. This strain serves as a control for the B6.Cg-Tg(SOD1*G93A)dl1Gur/J mutant strain (Stock No. 002299).

Control Information

  Control
   Noncarrier female
   000664 C57BL/6J
   NOTE: This strain is the control strain for the C57BL/6J-TgN(SOD1-G93A)1Gurdl mutant strain (Stock No. 002299).
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Tg(SOD1)2Gur allele
002297   B6SJL-Tg(SOD1)2Gur/J
View Strains carrying   Tg(SOD1)2Gur     (1 strain)

View Strains carrying other alleles of SOD1     (9 strains)

Additional Web Information

Congenic Nomenclature

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms
      assigned by genotype

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

Tg(SOD1)2Gur/0

        involves: C57BL/6 * SJL
  • nervous system phenotype
  • abnormal spinal cord morphology (MGI Ref ID J:76718)
    • develop neurofilament-rich spheroids in the spinal cords at much later time (132 days of age) points than Tg(SOD1-G93A)1Gur mutants, however do not appear to develop motor neuron disease

Tg(SOD1)2Gur/0

        involves: C57BL/6 * CBA * SJL
  • nervous system phenotype
  • abnormal oligodendrocyte morphology (MGI Ref ID J:134095)
    • ubiquinated SOD1 aggregates accumulate in oligodendrocytes of spinal cords of aged mice (>70 weeks) but a much lower density than in double transgenic T3/SOD1 animals
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)2Gur
Allele Name transgene insertion 2, Mark E Gurney
Allele Type Transgenic (random, expressed)
Common Name(s) N1029; N29; WT SOD1; tg-SOD1;
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 Expression level of the wild-type transgene in transgenic animals is comparable to that in transgenic mice carrying the original highest expressing line Tg(SOD1*G93A)2Gur.
Molecular Note The transgene contains the normal allele of the human superoxide dismutase 1 gene (SOD1). Transgene expression in brain was confirmed by enzyme immunoassay using antibodies that recognize human SOD1 protein. [MGI Ref ID J:32665]
 

Genotyping

Genotyping Information

Genotyping Protocols

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

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]

Tg(SOD1)2Gur related

Alexianu ME; Kozovska M; Appel SH. 2001. Immune reactivity in a mouse model of familial ALS correlates with disease progression. Neurology 57(7):1282-9. [PubMed: 11591849]  [MGI Ref ID J:78936]

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]

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]

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]

Bucher S; Braunstein KE; Niessen HG; Kaulisch T; Neumaier M; Boeckers TM; Stiller D; Ludolph AC. 2007. Vacuolization correlates with spin-spin relaxation time in motor brainstem nuclei and behavioural tests in the transgenic G93A-SOD1 mouse model of ALS. Eur J Neurosci 26(7):1895-901. [PubMed: 17868365]  [MGI Ref ID J:127265]

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]

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]

Craven PA; Melhem MF; Phillips SL; DeRubertis FR. 2001. Overexpression of Cu2+/Zn2+ superoxide dismutase protects against early diabetic glomerular injury in transgenic mice. Diabetes 50(9):2114-25. [PubMed: 11522679]  [MGI Ref ID J:107377]

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]

De Vos KJ; Chapman AL; Tennant ME; Manser C; Tudor EL; Lau KF; Brownlees J; Ackerley S; Shaw PJ; McLoughlin DM; Shaw CE; Leigh PN; Miller CC; Grierson AJ. 2007. Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content. Hum Mol Genet 16(22):2720-8. [PubMed: 17725983]  [MGI Ref ID J:129976]

De Winter F; Vo T; Stam FJ; Wisman LA; Bar PR; Niclou SP; van Muiswinkel FL; Verhaagen J. 2006. The expression of the chemorepellent Semaphorin 3A is selectively induced in terminal Schwann cells of a subset of neuromuscular synapses that display limited anatomical plasticity and enhanced vulnerability in motor neuron disease. Mol Cell Neurosci 32(1-2):102-17. [PubMed: 16677822]  [MGI Ref ID J:111941]

DeRubertis FR; Craven PA; Melhem MF; Salah EM. 2004. Attenuation of renal injury in db/db mice overexpressing superoxide dismutase: evidence for reduced superoxide-nitric oxide interaction. Diabetes 53(3):762-8. [PubMed: 14988262]  [MGI Ref ID J:88385]

Deng HX; Shi Y; Furukawa Y; Zhai H; Fu R; Liu E; Gorrie GH; Khan MS; Hung WY; Bigio EH; Lukas T; Dal Canto MC; O'Halloran TV; Siddique T. 2006. Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria. Proc Natl Acad Sci U S A 103(18):7142-7. [PubMed: 16636275]  [MGI Ref ID J:109458]

Dewil M; Schurmans C; Starckx S; Opdenakker G; Van Den Bosch L; Robberecht W. 2005. Role of matrix metalloproteinase-9 in a mouse model for amyotrophic lateral sclerosis. Neuroreport 16(4):321-4. [PubMed: 15729130]  [MGI Ref ID J:103535]

Dewil M; dela Cruz VF; Van Den Bosch L; Robberecht W. 2007. Inhibition of p38 mitogen activated protein kinase activation and mutant SOD1(G93A)-induced motor neuron death. Neurobiol Dis 26(2):332-41. [PubMed: 17346981]  [MGI Ref ID J:134849]

Di Giorgio FP; Carrasco MA; Siao MC; Maniatis T; Eggan K. 2007. Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model. Nat Neurosci 10(5):608-14. [PubMed: 17435754]  [MGI Ref ID J:121861]

Elliott JL. 2001. Cytokine upregulation in a murine model of familial amyotrophic lateral sclerosis. Brain Res Mol Brain Res 95(1-2):172-8. [PubMed: 11687290]  [MGI Ref ID J:72854]

Gal J; Strom AL; Kilty R; Zhang F; Zhu H. 2007. p62 accumulates and enhances aggregate formation in model systems of familial amyotrophic lateral sclerosis. J Biol Chem 282(15):11068-77. [PubMed: 17296612]  [MGI Ref ID J:121167]

Geracitano R; Paolucci E; Prisco S; Guatteo E; Zona C; Longone P; Ammassari-Teule M; Bernardi G; Berretta N; Mercuri NB. 2003. Altered long-term corticostriatal synaptic plasticity in transgenic mice overexpressing human CU/ZN superoxide dismutase (GLY(93)-->ALA) mutation. Neuroscience 118(2):399-408. [PubMed: 12699776]  [MGI Ref ID J:109423]

Gibb SL; Boston-Howes W; Lavina ZS; Gustincich S; Brown RH Jr; Pasinelli P; Trotti D. 2007. A caspase-3-cleaved fragment of the glial glutamate transporter EAAT2 is sumoylated and targeted to promyelocytic leukemia nuclear bodies in mutant SOD1-linked amyotrophic lateral sclerosis. J Biol Chem 282(44):32480-90. [PubMed: 17823119]  [MGI Ref ID J:126836]

Gilchrist CA; Gray DA; Stieber A; Gonatas NK; Kopito RR. 2005. Effect of ubiquitin expression on neuropathogenesis in a mouse model of familial amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol 31(1):20-33. [PubMed: 15634228]  [MGI Ref ID J:128568]

Gowing G; Philips T; Van Wijmeersch B; Audet JN; Dewil M; Van Den Bosch L; Billiau AD; Robberecht W; Julien JP. 2008. Ablation of proliferating microglia does not affect motor neuron degeneration in amyotrophic lateral sclerosis caused by mutant superoxide dismutase. J Neurosci 28(41):10234-44. [PubMed: 18842883]  [MGI Ref ID J:141126]

Guatteo E; Carunchio I; Pieri M; Albo F; Canu N; Mercuri NB; Zona C. 2007. Altered calcium homeostasis in motor neurons following AMPA receptor but not voltage-dependent calcium channels' activation in a genetic model of amyotrophic lateral sclerosis. Neurobiol Dis 28(1):90-100. [PubMed: 17706428]  [MGI Ref ID J:134834]

Harraz MM; Marden JJ; Zhou W; Zhang Y; Williams A; Sharov VS; Nelson K; Luo M; Paulson H; Schoneich C; Engelhardt JF. 2008. SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model. J Clin Invest 118(2):659-70. [PubMed: 18219391]  [MGI Ref ID J:131850]

Hegedus J; Putman CT; Gordon T. 2007. Time course of preferential motor unit loss in the SOD1 G93A mouse model of amyotrophic lateral sclerosis. Neurobiol Dis 28(2):154-64. [PubMed: 17766128]  [MGI Ref ID J:134803]

Jaarsma D; Haasdijk ED; Grashorn JA; Hawkins R; van Duijn W; Verspaget HW; London J; Holstege JC. 2000. Human Cu/Zn superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motoneuron death and accelerates motoneuron disease in mice expressing a familial amyotrophic lateral sclerosis mutant SOD1. Neurobiol Dis 7(6 Pt B):623-43. [PubMed: 11114261]  [MGI Ref ID J:66700]

Jaarsma D; Teuling E; Haasdijk ED; De Zeeuw CI; Hoogenraad CC. 2008. Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice. J Neurosci 28(9):2075-88. [PubMed: 18305242]  [MGI Ref ID J:134095]

Kawamata H; Magrane J; Kunst C; King MP; Manfredi G. 2008. Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria. J Biol Chem 283(42):28321-8. [PubMed: 18715867]  [MGI Ref ID J:142289]

Kikuchi H; Almer G; Yamashita S; Guegan C; Nagai M; Xu Z; Sosunov AA; McKhann GM 2nd; Przedborski S. 2006. Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model. Proc Natl Acad Sci U S A 103(15):6025-30. [PubMed: 16595634]  [MGI Ref ID J:108290]

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]

Kong J; Xu Z. 1999. Peripheral axotomy slows motoneuron degeneration in a transgenic mouse line expressing mutant SOD1 G93A. J Comp Neurol 412(2):373-80. [PubMed: 10441762]  [MGI Ref ID J:56912]

Kruidenier L; van Meeteren ME; Kuiper I; Jaarsma D; Lamers CB; Zijlstra FJ; Verspaget HW. 2003. Attenuated mild colonic inflammation and improved survival from severe DSS-colitis of transgenic Cu/Zn-SOD mice. Free Radic Biol Med 34(6):753-65. [PubMed: 12633752]  [MGI Ref ID J:82695]

Kunishige M; Hill KA; Riemer AM; Farwell KD; Halangoda A; Heinmoller E; Moore SR; Turner DM; Sommer SS. 2001. Mutation frequency is reduced in the cerebellum of Big Blue mice overexpressing a human wild type SOD1 gene. Mutat Res 473(2):139-49. [PubMed: 11166032]  [MGI Ref ID J:126863]

Larkin J; Clayton MM; Liu J; Feitelson MA. 2001. Chronic ethanol consumption stimulates hepatitis B virus gene expression and replication in transgenic mice. Hepatology 34(4 Pt 1):792-7. [PubMed: 11584377]  [MGI Ref ID J:106512]

Levine JB; Kong J; Nadler M; Xu Z. 1999. Astrocytes interact intimately with degenerating motor neurons in mouse amyotrophic lateral sclerosis (ALS). Glia 28(3):215-24. [PubMed: 10559780]  [MGI Ref ID J:59666]

Liu D; Wen J; Liu J; Li L. 1999. The roles of free radicals in amyotrophic lateral sclerosis: reactive oxygen species and elevated oxidation of protein, DNA, and membrane phospholipids. FASEB J 13(15):2318-28. [PubMed: 10593879]  [MGI Ref ID J:58839]

Liu JQ; Zelko IN; Folz RJ. 2004. Reoxygenation-induced constriction in murine coronary arteries: the role of endothelial NADPH oxidase (gp91phox) and intracellular superoxide. J Biol Chem 279(23):24493-7. [PubMed: 15070892]  [MGI Ref ID J:123986]

Lu L; Zheng L; Viera L; Suswam E; Li Y; Li X; Estevez AG; King PH. 2007. Mutant Cu/Zn-superoxide dismutase associated with amyotrophic lateral sclerosis destabilizes vascular endothelial growth factor mRNA and downregulates its expression. J Neurosci 27(30):7929-38. [PubMed: 17652584]  [MGI Ref ID J:123248]

Martin LJ; Chen K; Liu Z. 2005. Adult motor neuron apoptosis is mediated by nitric oxide and Fas death receptor linked by DNA damage and p53 activation. J Neurosci 25(27):6449-59. [PubMed: 16000635]  [MGI Ref ID J:99428]

Mattiazzi M; D'Aurelio M; Gajewski CD; Martushova K; Kiaei M; Beal MF; Manfredi G. 2002. Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J Biol Chem 277(33):29626-33. [PubMed: 12050154]  [MGI Ref ID J:132838]

Morimoto N; Nagai M; Ohta Y; Miyazaki K; Kurata T; Morimoto M; Murakami T; Takehisa Y; Ikeda Y; Kamiya T; Abe K. 2007. Increased autophagy in transgenic mice with a G93A mutant SOD1 gene. Brain Res 1167:112-7. [PubMed: 17689501]  [MGI Ref ID J:124927]

Niessen HG; Angenstein F; Sander K; Kunz WS; Teuchert M; Ludolph AC; Heinze HJ; Scheich H; Vielhaber S. 2006. In vivo quantification of spinal and bulbar motor neuron degeneration in the G93A-SOD1 transgenic mouse model of ALS by T2 relaxation time and apparent diffusion coefficient. Exp Neurol 201(2):293-300. [PubMed: 16740261]  [MGI Ref ID J:114432]

Niessen HG; Debska-Vielhaber G; Sander K; Angenstein F; Ludolph AC; Hilfert L; Willker W; Leibfritz D; Heinze HJ; Kunz WS; Vielhaber S. 2007. Metabolic progression markers of neurodegeneration in the transgenic G93A-SOD1 mouse model of amyotrophic lateral sclerosis. Eur J Neurosci 25(6):1669-77. [PubMed: 17432958]  [MGI Ref ID J:122876]

Puttaparthi K; Wojcik C; Rajendran B; DeMartino GN; Elliott JL. 2003. Aggregate formation in the spinal cord of mutant SOD1 transgenic mice is reversible and mediated by proteasomes. J Neurochem 87(4):851-60. [PubMed: 14622116]  [MGI Ref ID J:86631]

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]

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]

Thireau J; Poisson D; Zhang BL; Gillet L; Le Pecheur M; Andres C; London J; Babuty D. 2008. Increased heart rate variability in mice overexpressing the Cu/Zn superoxide dismutase. Free Radic Biol Med 45(4):396-403. [PubMed: 18513493]  [MGI Ref ID J:137769]

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]

Vlug AS; Teuling E; Haasdijk ED; French P; Hoogenraad CC; Jaarsma D. 2005. ATF3 expression precedes death of spinal motoneurons in amyotrophic lateral sclerosis-SOD1 transgenic mice and correlates with c-Jun phosphorylation, CHOP expression, somato-dendritic ubiquitination and Golgi fragmentation. Eur J Neurosci 22(8):1881-94. [PubMed: 16262628]  [MGI Ref ID J:102918]

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]

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]

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]

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]

Xu W; Chi L; Row BW; Xu R; Ke Y; Xu B; Luo C; Kheirandish L; Gozal D; Liu R. 2004. Increased oxidative stress is associated with chronic intermittent hypoxia-mediated brain cortical neuronal cell apoptosis in a mouse model of sleep apnea. Neuroscience 126(2):313-23. [PubMed: 15207349]  [MGI Ref ID J:91264]

Zhao P; Ignacio S; Beattie EC; Abood ME. 2008. Altered presymptomatic AMPA and cannabinoid receptor trafficking in motor neurons of ALS model mice: implications for excitotoxicity. Eur J Neurosci 27(3):572-9. [PubMed: 18279310]  [MGI Ref ID J:132266]

Zhao Z; Lange DJ; Voustianiouk A; MacGrogan D; Ho L; Suh J; Humala N; Thiyagarajan M; Wang J; Pasinetti GM. 2006. A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis. BMC Neurosci 7:29. [PubMed: 16584562]  [MGI Ref ID J:111122]

Zona C; Pieri M; Carunchio I. 2006. Voltage-dependent sodium channels in spinal cord motor neurons display rapid recovery from fast inactivation in a mouse model of amyotrophic lateral sclerosis. J Neurophysiol 96(6):3314-22. [PubMed: 16899637]  [MGI Ref ID J:136461]

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

Colony Maintenance

Breeding & HusbandryWhen held in a live colony, this strain is maintained by mating hemizygous male or female transgenic mice to C57LB/6J mice. Expected coat color from breeding:Black
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
Price (US dollars $)
Cryorecovery Fee $1900.00
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.

Additional Supply Details

Pricing for International shipping destinations View USA Canada and Mexico pricing
Price (US dollars $)
Cryorecovery Fee $2470.00
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.

Additional Supply Details

Supply Details

Standard SupplyCryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
Supply Notes
  • Cryorecovery - Standard.
    At least two mice that carry the mutation (if it is a mutant strain) will be provided. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotypes and genders are needed. IMPORTANT NOTE: The genotypes of the 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 for possible genotypes for this specific strain. Animals typically ship within 13 to 16 weeks from your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will typically ship within 25 weeks.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 or 1-207-288-5845.

  • This strain is included in the Induced Mutant Resource Colony collection.
  • Genomic DNA is available for this strain from the Mouse DNA Resource.

Control Information

  Control
   Noncarrier female
   000664 C57BL/6J
   NOTE: This strain is the control strain for the C57BL/6J-TgN(SOD1-G93A)1Gurdl mutant strain (Stock No. 002299).
 
  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.
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Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
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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.

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General inquiries

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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.”

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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, products and services. In addition, special terms and conditions of sale of certain MICE, products and services may be set forth separately in The Jackson Laboratory 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 The Jackson Laboratory, 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 The Jackson Laboratory, 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 services by The Jackson Laboratory.


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