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Gene Ontology Classifications
superoxide dismutase 2, mitochondrial

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GO curators for mouse genes have assigned the following annotations to the gene product of Sod2. (This text reflects annotations as of Tuesday, May 26, 2015.) MGI curation of this mouse gene is considered complete, including annotations derived from the biomedical literature as of August 1, 2008. If you know of any additional information regarding this mouse gene please let us know. Please supply mouse gene symbol and a PubMed ID.
Summary from NCBI RefSeq

[Summary is not available for the mouse gene. This summary is for the human ortholog.] This gene is a member of the iron/manganese superoxide dismutase family. It encodes a mitochondrial protein that forms a homotetramer and binds one manganese ion per subunit. This protein binds to the superoxide byproducts of oxidative phosphorylation and converts them to hydrogen peroxide and diatomic oxygen. Mutations in this gene have been associated with idiopathic cardiomyopathy (IDC), premature aging, sporadic motor neuron disease, and cancer. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq, Jul 2008]
Summary text based on GO annotations supported by experimental evidence in mouse
Summary text based on GO annotations supported by experimental evidence in other organisms
Summary text based on GO annotations supported by structural data
Summary text for additional MGI annotations
  1. Asikainen TM et al. (2002) Increased sensitivity of homozygous Sod2 mutant mice to oxygen toxicity. Free Radic Biol Med, 32:175-86. (PubMed:11796207)
  2. Bakthavatchalu V et al. (2012) Manganese superoxide dismutase is a mitochondrial fidelity protein that protects Polgamma against UV-induced inactivation. Oncogene, 31:2129-39. (PubMed:21909133)
  3. Baumgart E et al. (2001) Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse). Am J Pathol, 159:1477-94. (PubMed:11583975)
  4. Bogunovic B et al. (2008) An unexpected functional link between lysosomal thiol reductase and mitochondrial manganese superoxide dismutase. J Biol Chem, 283:8855-62. (PubMed:18218638)
  5. Copin JC et al. (2001) Prolonged hypoxia during cell development protects mature manganese superoxide dismutase-deficient astrocytes from damage by oxidative stress. FASEB J, 15:525-34. (PubMed:11156968)
  6. Da Cruz S et al. (2003) Proteomic analysis of the mouse liver mitochondrial inner membrane. J Biol Chem, 278:41566-71. (PubMed:12865426)
  7. Danial NN et al. (2003) BAD and glucokinase reside in a mitochondrial complex that integrates glycolysis and apoptosis. Nature, 424:952-6. (PubMed:12931191)
  8. Diaz F et al. (2005) Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency. Hum Mol Genet, 14:2737-48. (PubMed:16103131)
  9. Friedman JS et al. (2004) SOD2-deficiency anemia: protein oxidation and altered protein expression reveal targets of damage, stress response, and antioxidant responsiveness. Blood, 104:2565-73. (PubMed:15205258)
  10. Fujimura M et al. (1999) Manganese superoxide dismutase mediates the early release of mitochondrial cytochrome C and subsequent DNA fragmentation after permanent focal cerebral ischemia in mice. J Neurosci, 19:3414-22. (PubMed:10212301)
  11. Ikegami T et al. (2002) Model mice for tissue-specific deletion of the manganese superoxide dismutase (MnSOD) gene. Biochem Biophys Res Commun, 296:729-36. (PubMed:12176043)
  12. Ke BX et al. (2012) Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathy. Proc Natl Acad Sci U S A, 109:6165-70. (PubMed:22474353)
  13. Khan JY et al. (2003) Developmental changes in murine brain antioxidant enzymes. Pediatr Res, 54:77-82. (PubMed:12646716)
  14. Kinugawa S et al. (2005) Limited exercise capacity in heterozygous manganese superoxide dismutase gene-knockout mice: roles of superoxide anion and nitric oxide. Circulation, 111:1480-6. (PubMed:15781740)
  15. Kokoszka JE et al. (2001) Increased mitochondrial oxidative stress in the Sod2 (+/-) mouse results in the age-related decline of mitochondrial function culminating in increased apoptosis. Proc Natl Acad Sci U S A, 98:2278-83. (PubMed:11226230)
  16. Lapointe J et al. (2009) Reversal of the mitochondrial phenotype and slow development of oxidative biomarkers of aging in long-lived Mclk1+/- mice. J Biol Chem, 284:20364-74. (PubMed:19478076)
  17. Lebovitz RM et al. (1996) Neurodegeneration, myocardial injury, and perinatal death in mitochondrial superoxide dismutase-deficient mice. Proc Natl Acad Sci U S A, 93:9782-7. (PubMed:8790408)
  18. Lechpammer S et al. (2005) Adipocyte differentiation in Sod2(-/-) and Sod2(+/+) murine bone marrow stromal cells is associated with low antioxidant pools. Exp Hematol, 33:1201-8. (PubMed:16219542)
  19. Lenart J et al. (2007) Deficiency of manganese superoxide dismutase in hepatocytes disrupts zonated gene expression in mouse liver. Arch Biochem Biophys, 462:238-44. (PubMed:17367743)
  20. Li Y et al. (1995) Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase. Nat Genet, 11:376-81. (PubMed:7493016)
  21. Lombard DB et al. (2007) Mammalian Sir2 homolog SIRT3 regulates global mitochondrial lysine acetylation. Mol Cell Biol, 27:8807-14. (PubMed:17923681)
  22. Martelli A et al. (2007) Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues. Hum Mol Genet, 16:2651-8. (PubMed:17597094)
  23. Mashimo M et al. (2013) ADP-ribosyl-acceptor hydrolase 3 regulates poly (ADP-ribose) degradation and cell death during oxidative stress. Proc Natl Acad Sci U S A, 110:18964-9. (PubMed:24191052)
  24. Melov S et al. (1999) Mitochondrial disease in superoxide dismutase 2 mutant mice. Proc Natl Acad Sci U S A, 96:846-51. (PubMed:9927656)
  25. Melov S et al. (1998) A novel neurological phenotype in mice lacking mitochondrial manganese superoxide dismutase [see comments] Nat Genet, 18:159-63. (PubMed:9462746)
  26. Misawa H et al. (2006) Conditional knockout of Mn superoxide dismutase in postnatal motor neurons reveals resistance to mitochondrial generated superoxide radicals. Neurobiol Dis, 23:169-77. (PubMed:16677818)
  27. Mootha VK et al. (2003) Integrated analysis of protein composition, tissue diversity, and gene regulation in mouse mitochondria. Cell, 115:629-40. (PubMed:14651853)
  28. Ohashi M et al. (2006) MnSOD deficiency increases endothelial dysfunction in ApoE-deficient mice. Arterioscler Thromb Vasc Biol, 26:2331-6. (PubMed:16873728)
  29. Oktay Y et al. (2007) Hypoxia-inducible factor 2alpha regulates expression of the mitochondrial aconitase chaperone protein frataxin. J Biol Chem, 282:11750-6. (PubMed:17322295)
  30. Pagliarini DJ et al. (2008) A mitochondrial protein compendium elucidates complex I disease biology. Cell, 134:112-23. (PubMed:18614015)
  31. Reddy VN et al. (2004) Effects of variation in superoxide dismutases (SOD) on oxidative stress and apoptosis in lens epithelium. Exp Eye Res, 79:859-68. (PubMed:15642323)
  32. Rodriguez-Iturbe B et al. (2007) Association of mitochondrial SOD deficiency with salt-sensitive hypertension and accelerated renal senescence. J Appl Physiol, 102:255-60. (PubMed:17023572)
  33. Sandbach JM et al. (2001) Ocular pathology in mitochondrial superoxide dismutase (Sod2)-deficient mice. Invest Ophthalmol Vis Sci, 42:2173-8. (PubMed:11527927)
  34. Szymura JM et al. (1981) Mouse mitochondrial superoxide dismutase locus is on chromosome 17. Immunogenetics, 14:231-40. (PubMed:7333654)
  35. Tsan MF et al. (1998) Susceptibility of heterozygous MnSOD gene-knockout mice to oxygen toxicity. Am J Respir Cell Mol Biol, 19:114-20. (PubMed:9651187)
  36. Van Remmen H et al. (2004) Multiple deficiencies in antioxidant enzymes in mice result in a compound increase in sensitivity to oxidative stress. Free Radic Biol Med, 36:1625-34. (PubMed:15182862)
  37. Van Remmen H et al. (1999) Characterization of the antioxidant status of the heterozygous manganese superoxide dismutase knockout mouse. Arch Biochem Biophys, 363:91-7. (PubMed:10049502)
  38. Vasilaki A et al. (2006) Genetic modification of the manganese superoxide dismutase/glutathione peroxidase 1 pathway influences intracellular ROS generation in quiescent, but not contracting, skeletal muscle cells. Free Radic Biol Med, 41:1719-25. (PubMed:17145560)
  39. Werner HB et al. (2007) Proteolipid protein is required for transport of sirtuin 2 into CNS myelin. J Neurosci, 27:7717-30. (PubMed:17634366)
  40. Yan C et al. (2005) Increased superoxide leads to decreased flow-induced dilation in resistance arteries of Mn-SOD-deficient mice. Am J Physiol Heart Circ Physiol, 288:H2225-31. (PubMed:15637112)
  41. Yao D et al. (2007) High glucose increases angiopoietin-2 transcription in microvascular endothelial cells through methylglyoxal modification of mSin3A. J Biol Chem, 282:31038-45. (PubMed:17670746)

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Gene Ontology Evidence Code Abbreviations:

  EXP Inferred from experiment
  IAS Inferred from ancestral sequence
  IBA Inferred from biological aspect of ancestor
  IBD Inferred from biological aspect of descendant
  IC Inferred by curator
  IDA Inferred from direct assay
  IEA Inferred from electronic annotation
  IGI Inferred from genetic interaction
  IKR Inferred from key residues
  IMP Inferred from mutant phenotype
  IMR Inferred from missing residues
  IPI Inferred from physical interaction
  IRD Inferred from rapid divergence
  ISS Inferred from sequence or structural similarity
  ISO Inferred from sequence orthology
  ISA Inferred from sequence alignment
  ISM Inferred from sequence model
  NAS Non-traceable author statement
  ND No biological data available
  RCA Reviewed computational analysis
  TAS Traceable author statement


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