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Type Mutant Strain; Transgenic; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Generation N?+12p Donating Investigator Charles Epstein, UCSF School of Medicine Appearance
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Related Genotype: a/aDescription
Transgenic mice are viable and express human SOD1. Transgenic mice express three times the normal level of SOD1 in the blood, brain, and fibroblasts. Homozygotes for the Tg(SOD1)3Cje transgene are fertile. Described in the literature as 218/3 denoting that the transgene insertion site is maps to Chr 3 (Shi, et al., 1994). Of the two lines (the other being C57BL/6-Tg(SOD1)10Cje/J, Stock No. 002628), this is the most widely used.Development
These strains were developed in the lab of Dr. Charles J. Epstein at the University of California at San Francisco School of Medicine. The transgene is present in multiple copies.
| Control | ||
|---|---|---|
| Noncarrier | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Strains carrying other alleles of SOD1
002298 B6.Cg-Tg(SOD1)2Gur/J 008342 B6.Cg-Tg(SOD1*G37R)42Dpr/J 008248 B6.Cg-Tg(SOD1*G85R)148Dwc/J 004435 B6.Cg-Tg(SOD1*G93A)1Gur/J 002299 B6.Cg-Tg(SOD1*G93A)dl1Gur/J 002297 B6SJL-Tg(SOD1)2Gur/J 002726 B6SJL-Tg(SOD1*G93A)1Gur/J 002300 B6SJL-Tg(SOD1*G93A)dl1Gur/J 002628 C57BL/6-Tg(SOD1)10Cje/J 008230 FVB(Cg)-Tg(Thy1-SOD1*G93A)T3Hgrd/J View Strains carrying other alleles of SOD1 (10 strains)
View Mammalian Phenotype Terms
Mammalian Phenotype Terms
assigned by genotype
Tg(SOD1)3Cje/0
C57BL/6-Tg(SOD1)3Cje/J
- hearing/vestibular/ear phenotype
- decreased brainstem auditory evoked potential (MGI Ref ID J:102419)
- over-expression of SOD1 does not protect against hearing loss except at 24 months of age, when hemizygous transgenic mice display slightly reduced ABR thresholds relative to age-matched C57BL/6 control mice
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Tg(SOD1)3Cje/0
involves: BALB/c * C57BL/6J * DBA/2
- cardiovascular system phenotype
- myocardial necrosis (MGI Ref ID J:89952)
- extent of myocardial necrosis following ischemia and reperfusion is similar to that observed in wild-type
- muscle phenotype
- myocardial necrosis (MGI Ref ID J:89952)
- extent of myocardial necrosis following ischemia and reperfusion is similar to that observed in wild-type
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)
Behavioral and Learning Defects
Metabolic Defects
| Allele Symbol | Tg(SOD1)3Cje | ||
|---|---|---|---|
| Allele Name | transgene insertion 3, Charles J Epstein | ||
| Allele Type | Transgenic (random, expressed) | ||
| Common Name(s) |
218/3;
Cu/ZnSOD-Tg;
CuZn SOD Tg;
SOD TgN;
SOD-TgN;
SOD1 TGHS/SF-218-3;
SOD1 TgHS/SF-213-3;
TgHS-218/3;
TgNSOD13Cje;
TgN[SOD1]3Cje, hSOD1 | ||
| Mutation Made By | Charles Epstein, UCSF School of Medicine | ||
| Strain of Origin | (BALB/c x C57BL/6J)F1 x (C57BL/6J x DBA/2)F1 | ||
| Expressed Gene | SOD1, superoxide dismutase 1, soluble, human | ||
| General Note |
Line 10 was also produced. Transgenic mice on a C57BL/6 background are viable; homozygotes are fertile. Transgenic mice express three times the normal level of SOD1 in the blood, brain, cochlea, and fibroblasts. | ||
| Molecular Note | The entire human SOD1 sequence was used for the transgene. Seven copies of the transgene inserted into Chromosome 3 (J:18645). Northern blot analysis of brain and liver from transgenic animals showed expression of 0.9 kb and 0.7 kb transcripts similar tothat in humans. Transgenic animals exhibited increased enzyme activity in nearly all tissues examined. [MGI Ref ID J:30458] | ||
Genotyping Protocols
Tg(SOD1), STD PCR, vers. 1
Helpful Links
Optimizing PCR Protocols
Epstein CJ; Avraham KB; Lovett M; Smith S; Elroy-Stein O; Rotman G; Bry C; Groner Y. 1987. Transgenic mice with increased Cu/Zn-superoxide dismutase activity: animal model of dosage effects in Down syndrome. Proc Natl Acad Sci U S A 84(22):8044-8. [PubMed: 2960971] [MGI Ref ID J:30458]
Jones SP; Hoffmeyer MR; Sharp BR; Ho YS; Lefer DJ. 2003. Role of intracellular antioxidant enzymes after in vivo myocardial ischemia and reperfusion. Am J Physiol Heart Circ Physiol 284(1):H277-82. [PubMed: 12485820] [MGI Ref ID J:89952]
Levy MA; Tsai YH; Reaume A; Bray TM. 2001. Cellular response of antioxidant metalloproteins in Cu/Zn SOD transgenic mice exposed to hyperoxia. Am J Physiol Lung Cell Mol Physiol 281(1):L172-82. [PubMed: 11404260] [MGI Ref ID J:70293]
Tg(SOD1)3Cje relatedAdachi T; Weisbrod RM; Pimentel DR; Ying J; Sharov VS; Schoneich C; Cohen RA. 2004. S-Glutathiolation by peroxynitrite activates SERCA during arterial relaxation by nitric oxide. Nat Med 10(11):1200-7. [PubMed: 15489859] [MGI Ref ID J:94015]
Avraham KB; Schickler M; Sapoznikov D; Yarom R; Groner Y. 1988. Down's syndrome: abnormal neuromuscular junction in tongue of transgenic mice with elevated levels of human Cu/Zn-superoxide dismutase. Cell 54(6):823-9. [PubMed: 2970304] [MGI Ref ID J:81720]
Cadet JL; Sheng P; Ali S; Rothman R; Carlson E; Epstein C. 1994. Attenuation of methamphetamine-induced neurotoxicity in copper/zinc superoxide dismutase transgenic mice. J Neurochem 62(1):380-3. [PubMed: 7505315] [MGI Ref ID J:78628]
Cardozo-Pelaez F; Song S; Parthasarathy A; Epstein CJ; Sanchez-Ramos J. 1998. Attenuation of age-dependent oxidative damage to DNA and protein in brainstem of Tg Cu/Zn SOD mice. Neurobiol Aging 19(4):311-6. [PubMed: 9733163] [MGI Ref ID J:129137]
Coling DE; Yu KC; Somand D; Satar B; Bai U; Huang TT; Seidman MD; Epstein CJ; Mhatre AN; Lalwani AK. 2003. Effect of SOD1 overexpression on age- and noise-related hearing loss. Free Radic Biol Med 34(7):873-80. [PubMed: 12654476] [MGI Ref ID J:82694]
Copin JC; Gasche Y; Chan PH. 2000. Overexpression of copper/zinc superoxide dismutase does not prevent neonatal lethality in mutant mice that lack manganese superoxide dismutase. Free Radic Biol Med 28(10):1571-6. [PubMed: 10927183] [MGI Ref ID J:115326]
Endo T; Nakagawa T; Iguchi F; Kita T; Okano T; Sha SH; Schacht J; Shiga A; Kim TS; Ito J. 2005. Elevation of superoxide dismutase increases acoustic trauma from noise exposure. Free Radic Biol Med 38(4):492-8. [PubMed: 15649651] [MGI Ref ID J:95541]
Fujimura M; Morita-Fujimura Y; Narasimhan P; Copin JC; Kawase M; Chan PH. 1999. Copper-zinc superoxide dismutase prevents the early decrease of apurinic/apyrimidinic endonuclease and subsequent DNA fragmentation after transient focal cerebral ischemia in mice. Stroke 30(11):2408-15. [PubMed: 10548678] [MGI Ref ID J:129259]
Fukada K; Nagano S; Satoh M; Tohyama C; Nakanishi T; Shimizu A; Yanagihara T; Sakoda S. 2001. Stabilization of mutant Cu/Zn superoxide dismutase (SOD1) protein by coexpressed wild SOD1 protein accelerates the disease progression in familial amyotrophic lateral sclerosis mice. Eur J Neurosci 14(12):2032-6. [PubMed: 11860498] [MGI Ref ID J:108078]
Gahtan E; Auerbach JM; Groner Y; Segal M. 1998. Reversible impairment of long-term potentiation in transgenic Cu/Zn-SOD mice. Eur J Neurosci 10(2):538-44. [PubMed: 9749716] [MGI Ref ID J:91720]
Horie Y; Wolf R; Flores SC; McCord JM; Epstein CJ; Granger DN. 1998. Transgenic mice with increased copper/zinc-superoxide dismutase activity are resistant to hepatic leukostasis and capillary no-reflow after gut ischemia/reperfusion. Circ Res 83(7):691-6. [PubMed: 9758638] [MGI Ref ID J:79504]
Huang CY; Fujimura M; Chang YY; Chan PH. 2001. Overexpression of copper-zinc superoxide dismutase attenuates acute activation of activator protein-1 after transient focal cerebral ischemia in mice. Stroke 32(3):741-7. [PubMed: 11239196] [MGI Ref ID J:127965]
Jayanthi S; Ladenheim B; Andrews AM; Cadet JL. 1999. Overexpression of human copper/zinc superoxide dismutase in transgenic mice attenuates oxidative stress caused by methylenedioxymethamphetamine (Ecstasy). Neuroscience 91(4):1379-87. [PubMed: 10391444] [MGI Ref ID J:57353]
Jones SP; Hoffmeyer MR; Sharp BR; Ho YS; Lefer DJ. 2003. Role of intracellular antioxidant enzymes after in vivo myocardial ischemia and reperfusion. Am J Physiol Heart Circ Physiol 284(1):H277-82. [PubMed: 12485820] [MGI Ref ID J:89952]
Kamsler A; Segal M. 2003. Paradoxical actions of hydrogen peroxide on long-term potentiation in transgenic superoxide dismutase-1 mice. J Neurosci 23(32):10359-67. [PubMed: 14614095] [MGI Ref ID J:86583]
Karliner JS; Honbo N; Epstein CJ; Xian M; Lau YF; Gray MO. 2000. Neonatal mouse cardiac myocytes exhibit cardioprotection induced by hypoxic and pharmacologic preconditioning and by transgenic overexpression of human Cu/Zn superoxide dismutase. J Mol Cell Cardiol 32(10):1779-86. [PubMed: 11013122] [MGI Ref ID J:127812]
Keithley EM; Canto C; Zheng QY; Wang X; Fischel-Ghodsian N; Johnson KR. 2005. Cu/Zn superoxide dismutase and age-related hearing loss. Hear Res 209(1-2):76-85. [PubMed: 16055286] [MGI Ref ID J:102419]
Krieglstein CF; Cerwinka WH; Laroux FS; Salter JW; Russell JM; Schuermann G; Grisham MB; Ross CR; Granger DN. 2001. Regulation of murine intestinal inflammation by reactive metabolites of oxygen and nitrogen: divergent roles of superoxide and nitric oxide. J Exp Med 194(9):1207-18. [PubMed: 11696587] [MGI Ref ID J:134388]
Mori M; Stokes KY; Vowinkel T; Watanabe N; Elrod JW; Harris NR; Lefer DJ; Hibi T; Granger DN. 2005. Colonic blood flow responses in experimental colitis: time course and underlying mechanisms. Am J Physiol Gastrointest Liver Physiol 289(6):G1024-9. [PubMed: 16081759] [MGI Ref ID J:104795]
Murakami K; Kondo T; Epstein CJ; Chan PH. 1997. Overexpression of CuZn-superoxide dismutase reduces hippocampal injury after global ischemia in transgenic mice. Stroke 28(9):1797-804. [PubMed: 9303028] [MGI Ref ID J:43889]
Narasimhan P; Fujimura M; Noshita N; Chan PH. 2003. Role of superoxide in poly(ADP-ribose) polymerase upregulation after transient cerebral ischemia. Brain Res Mol Brain Res 113(1-2):28-36. [PubMed: 12750003] [MGI Ref ID J:83639]
Noshita N; Sugawara T; Hayashi T; Lewen A; Omar G; Chan PH. 2002. Copper/zinc superoxide dismutase attenuates neuronal cell death by preventing extracellular signal-regulated kinase activation after transient focal cerebral ischemia in mice. J Neurosci 22(18):7923-30. [PubMed: 12223545] [MGI Ref ID J:79147]
Peter Y; Rotman G; Lotem J; Elson A; Shiloh Y; Groner Y. 2001. Elevated Cu/Zn-SOD exacerbates radiation sensitivity and hematopoietic abnormalities of Atm-deficient mice. EMBO J 20(7):1538-46. [PubMed: 11285218] [MGI Ref ID J:68771]
Petnehazy T; Cooper D; Stokes KY; Russell J; Wood KC; Granger DN. 2006. Angiotensin II type 1 receptors and the intestinal microvascular dysfunction induced by ischemia and reperfusion. Am J Physiol Gastrointest Liver Physiol 290(6):G1203-10. [PubMed: 16469824] [MGI Ref ID J:111088]
Petrache I; Medler TR; Richter AT; Kamocki K; Chukwueke U; Zhen L; Gu Y; Adamowicz J; Schweitzer KS; Hubbard WC; Berdyshev EV; Lungarella G; Tuder RM. 2008. Superoxide dismutase protects against apoptosis and alveolar enlargement induced by ceramide. Am J Physiol Lung Cell Mol Physiol 295(1):L44-53. [PubMed: 18441093] [MGI Ref ID J:138536]
Przedborski S; Kostic V; Jackson-Lewis V; Naini AB; Simonetti S; Fahn S; Carlson E; Epstein CJ; Cadet JL. 1992. Transgenic mice with increased Cu/Zn-superoxide dismutase activity are resistant to N-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-induced neurotoxicity. J Neurosci 12(5):1658-67. [PubMed: 1578260] [MGI Ref ID J:81721]
Saito A; Hayashi T; Okuno S; Nishi T; Chan PH. 2006. Modulation of proline-rich akt substrate survival signaling pathways by oxidative stress in mouse brains after transient focal cerebral ischemia. Stroke 37(2):513-7. [PubMed: 16397181] [MGI Ref ID J:116422]
Saito A; Hayashi T; Okuno S; Nishi T; Chan PH. 2004. Modulation of the Omi/HtrA2 signaling pathway after transient focal cerebral ischemia in mouse brains that overexpress SOD1. Brain Res Mol Brain Res 127(1-2):89-95. [PubMed: 15306124] [MGI Ref ID J:134596]
Saito A; Hayashi T; Okuno S; Nishi T; Chan PH. 2004. Oxidative stress affects the integrin-linked kinase signaling pathway after transient focal cerebral ischemia. Stroke 35(11):2560-5. [PubMed: 15472100] [MGI Ref ID J:134752]
Sarco DP; Becker J; Palmer C; Sheldon RA; Ferriero DM. 2000. The neuroprotective effect of deferoxamine in the hypoxic-ischemic immature mouse brain. Neurosci Lett 282(1-2):113-6. [PubMed: 10713409] [MGI Ref ID J:61282]
Schickler M; Knobler H; Avraham KB; Elroy-Stein O; Groner Y. 1989. Diminished serotonin uptake in platelets of transgenic mice with increased Cu/Zn-superoxide dismutase activity. EMBO J 8(5):1385-92. [PubMed: 2527742] [MGI Ref ID J:79913]
Shi YP; Huang TT; Carlson EJ; Epstein CJ. 1994. The mapping of transgenes by fluorescence in situ hybridization on G-banded mouse chromosomes. Mamm Genome 5(6):337-41. [PubMed: 8043947] [MGI Ref ID J:18645]
Stokes KY; Clanton EC; Russell JM; Ross CR; Granger DN. 2001. NAD(P)H oxidase-derived superoxide mediates hypercholesterolemia-induced leukocyte-endothelial cell adhesion. Circ Res 88(5):499-505. [PubMed: 11249873] [MGI Ref ID J:115583]
Stokes KY; Russell JM; Jennings MH; Alexander JS; Granger DN. 2007. Platelet-associated NAD(P)H oxidase contributes to the thrombogenic phenotype induced by hypercholesterolemia. Free Radic Biol Med 43(1):22-30. [PubMed: 17561090] [MGI Ref ID J:122384]
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]
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]
Visser JE; Smith DW; Moy SS; Breese GR; Friedmann T; Rothstein JD; Jinnah HA. 2002. Oxidative stress and dopamine deficiency in a genetic mouse model of Lesch--Nyhan disease. Brain Res Dev Brain Res 133(2):127-39. [PubMed: 11882343] [MGI Ref ID J:75479]
Weinzierl M; Mautes AE; Whetstone W; Lin Y; Noble-Haeusslein LJ. 2004. Endothelin-mediated induction of heme oxygenase-1 in the spinal cord is attenuated in transgenic mice overexpressing superoxide dismutase. Brain Res 1030(1):125-32. [PubMed: 15567344] [MGI Ref ID J:94158]
Wood KC; Hebbel RP; Granger DN. 2005. Endothelial cell NADPH oxidase mediates the cerebral microvascular dysfunction in sickle cell transgenic mice. FASEB J 19(8):989-91. [PubMed: 15923406] [MGI Ref ID J:128246]
Xiong Y; Shie FS; Zhang J; Lee CP; Ho YS. 2005. Prevention of mitochondrial dysfunction in post-traumatic mouse brain by superoxide dismutase. J Neurochem 95(3):732-44. [PubMed: 16248885] [MGI Ref ID J:136792]
Colony Maintenance
Diet Information LabDiet® 5K52/5K67
| Pricing for USA, Canada and Mexico shipping destinations |
|
Animals Provided
Price (US dollars $) Cryorecovery Fee $1900.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
| Pricing for International shipping destinations |
|
Animals Provided
Price (US dollars $) Cryorecovery Fee $2470.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
| Standard Supply | Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information. |
|---|---|
| Supply Notes |
|
| Control | ||
|---|---|---|
| Noncarrier | ||
| 000664 C57BL/6J | ||
| 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. | ||
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Fax: 207.288.6150
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| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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