You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Identification
HMDB Protein ID HMDBP01956
Secondary Accession Numbers
  • 7375
Name Superoxide dismutase [Mn], mitochondrial
Synonyms Not Available
Gene Name SOD2
Protein Type Unknown
Biological Properties
General Function Involved in superoxide dismutase activity
Specific Function Destroys superoxide anion radicals which are normally produced within the cells and which are toxic to biological systems.
Pathways
  • Degradation of Superoxides
  • Huntington's disease
  • Peroxisome
Reactions
Superoxide + Hydrogen Ion → Oxygen + Hydrogen peroxide details
GO Classification
Biological Process
oxygen homeostasis
locomotory behavior
cellular response to ethanol
neuron development
response to cadmium ion
response to zinc ion
response to electrical stimulus
negative regulation of neuron apoptotic process
age-dependent response to reactive oxygen species
erythrophore differentiation
negative regulation of fibroblast proliferation
negative regulation of fat cell differentiation
regulation of catalytic activity
response to silicon dioxide
vasodilation by acetylcholine involved in regulation of systemic arterial blood pressure
response to lipopolysaccharide
response to gamma radiation
response to L-ascorbic acid
regulation of mitochondrial membrane potential
iron ion homeostasis
liver development
response to drug
hydrogen peroxide biosynthetic process
glutathione metabolic process
heart development
hemopoiesis
detection of oxygen
positive regulation of nitric oxide biosynthetic process
regulation of transcription from RNA polymerase II promoter
superoxide anion generation
response to activity
response to selenium ion
protein homotetramerization
negative regulation of cell proliferation
release of cytochrome c from mitochondria
removal of superoxide radicals
response to hyperoxia
response to hydrogen peroxide
response to axon injury
response to hypoxia
response to manganese ion
respiratory electron transport chain
post-embryonic development
Cellular Component
mitochondrial matrix
mitochondrial nucleoid
mitochondrial inner membrane
Function
ion binding
cation binding
metal ion binding
binding
antioxidant activity
superoxide dismutase activity
Molecular Function
oxygen binding
manganese ion binding
superoxide dismutase activity
DNA binding
Process
metabolic process
cellular metabolic process
oxidation reduction
oxygen and reactive oxygen species metabolic process
superoxide metabolic process
Cellular Location
  1. Mitochondrion matrix
Gene Properties
Chromosome Location 6
Locus 6q25.3
SNPs SOD2
Gene Sequence
>669 bp
ATGTTGAGCCGGGCAGTGTGCGGCACCAGCAGGCAGCTGGCTCCGGCTTTGGGGTATCTG
GGCTCCAGGCAGAAGCACAGCCTCCCCGACCTGCCCTACGACTACGGCGCCCTGGAACCT
CACATCAACGCGCAGATCATGCAGCTGCACCACAGCAAGCACCACGCGGCCTACGTGAAC
AACCTGAACGTCAACGAGGAGAAGTACCAGGAGGCGTTGGCCAAGGGAGATGTTACAGCC
CAGATAGCTCTTCAGCCTGCACTGAAGTTCAATGGTGGTGGTCATATCAATCATAGCATT
TTCTGGACAAACCTCAGCCCTAACGGTGGTGGAGAACCCAAAGGGGAGTTGCTGGAAGCC
ATCAAACGTGACTTTGGTTCCTTTGACAAGTTTAAGGAGAAGCTGACGGCTGCATCTGTT
GGTGTCCAAGGCTCAGGTTGGGGTTGGCTTGGTTTCAATAAGGAACGGGGACACTTACAA
ATTGCTGCTTGTCCAAATCAGGATCCACTGCAAGGAACAACAGGCCTTATTCCACTGCTG
GGGATTGATGTGTGGGAGCACGCTTACTACCTTCAGTATAAAAATGTCAGGCCTGATTAT
CTAAAAGCTATTTGGAATGTAATCAACTGGGAGAATGTAACTGAAAGATACATGGCTTGC
AAAAAGTAA
Protein Properties
Number of Residues 222
Molecular Weight 24750.015
Theoretical pI 8.256
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Superoxide dismutase [Mn], mitochondrial
MLSRAVCGTSRQLAPALGYLGSRQKHSLPDLPYDYGALEPHINAQIMQLHHSKHHAAYVN
NLNVTEEKYQEALAKGDVTAQIALQPALKFNGGGHINHSIFWTNLSPNGGGEPKGELLEA
IKRDFGSFDKFKEKLTAASVGVQGSGWGWLGFNKERGHLQIAACPNQDPLQGTTGLIPLL
GIDVWEHAYYLQYKNVRPDYLKAIWNVINWENVTERYMACKK
GenBank ID Protein 34709
UniProtKB/Swiss-Prot ID P04179
UniProtKB/Swiss-Prot Entry Name SODM_HUMAN
PDB IDs
GenBank Gene ID X59445
GeneCard ID SOD2
GenAtlas ID SOD2
HGNC ID HGNC:11180
References
General References
  1. Mungall AJ, Palmer SA, Sims SK, Edwards CA, Ashurst JL, Wilming L, Jones MC, Horton R, Hunt SE, Scott CE, Gilbert JG, Clamp ME, Bethel G, Milne S, Ainscough R, Almeida JP, Ambrose KD, Andrews TD, Ashwell RI, Babbage AK, Bagguley CL, Bailey J, Banerjee R, Barker DJ, Barlow KF, Bates K, Beare DM, Beasley H, Beasley O, Bird CP, Blakey S, Bray-Allen S, Brook J, Brown AJ, Brown JY, Burford DC, Burrill W, Burton J, Carder C, Carter NP, Chapman JC, Clark SY, Clark G, Clee CM, Clegg S, Cobley V, Collier RE, Collins JE, Colman LK, Corby NR, Coville GJ, Culley KM, Dhami P, Davies J, Dunn M, Earthrowl ME, Ellington AE, Evans KA, Faulkner L, Francis MD, Frankish A, Frankland J, French L, Garner P, Garnett J, Ghori MJ, Gilby LM, Gillson CJ, Glithero RJ, Grafham DV, Grant M, Gribble S, Griffiths C, Griffiths M, Hall R, Halls KS, Hammond S, Harley JL, Hart EA, Heath PD, Heathcott R, Holmes SJ, Howden PJ, Howe KL, Howell GR, Huckle E, Humphray SJ, Humphries MD, Hunt AR, Johnson CM, Joy AA, Kay M, Keenan SJ, Kimberley AM, King A, Laird GK, Langford C, Lawlor S, Leongamornlert DA, Leversha M, Lloyd CR, Lloyd DM, Loveland JE, Lovell J, Martin S, Mashreghi-Mohammadi M, Maslen GL, Matthews L, McCann OT, McLaren SJ, McLay K, McMurray A, Moore MJ, Mullikin JC, Niblett D, Nickerson T, Novik KL, Oliver K, Overton-Larty EK, Parker A, Patel R, Pearce AV, Peck AI, Phillimore B, Phillips S, Plumb RW, Porter KM, Ramsey Y, Ranby SA, Rice CM, Ross MT, Searle SM, Sehra HK, Sheridan E, Skuce CD, Smith S, Smith M, Spraggon L, Squares SL, Steward CA, Sycamore N, Tamlyn-Hall G, Tester J, Theaker AJ, Thomas DW, Thorpe A, Tracey A, Tromans A, Tubby B, Wall M, Wallis JM, West AP, White SS, Whitehead SL, Whittaker H, Wild A, Willey DJ, Wilmer TE, Wood JM, Wray PW, Wyatt JC, Young L, Younger RM, Bentley DR, Coulson A, Durbin R, Hubbard T, Sulston JE, Dunham I, Rogers J, Beck S: The DNA sequence and analysis of human chromosome 6. Nature. 2003 Oct 23;425(6960):805-11. [PubMed:14574404 ]
  2. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [PubMed:14702039 ]
  3. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  4. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [PubMed:19608861 ]
  5. Corbett JM, Wheeler CH, Baker CS, Yacoub MH, Dunn MJ: The human myocardial two-dimensional gel protein database: update 1994. Electrophoresis. 1994 Nov;15(11):1459-65. [PubMed:7895732 ]
  6. Rasmussen RK, Ji H, Eddes JS, Moritz RL, Reid GE, Simpson RJ, Dorow DS: Two-dimensional electrophoretic analysis of human breast carcinoma proteins: mapping of proteins that bind to the SH3 domain of mixed lineage kinase MLK2. Electrophoresis. 1997 Mar-Apr;18(3-4):588-98. [PubMed:9150946 ]
  7. Kovalyov LI, Shishkin SS, Efimochkin AS, Kovalyova MA, Ershova ES, Egorov TA, Musalyamov AK: The major protein expression profile and two-dimensional protein database of human heart. Electrophoresis. 1995 Jul;16(7):1160-9. [PubMed:7498159 ]
  8. Xu S, Ying J, Jiang B, Guo W, Adachi T, Sharov V, Lazar H, Menzoian J, Knyushko TV, Bigelow D, Schoneich C, Cohen RA: Detection of sequence-specific tyrosine nitration of manganese SOD and SERCA in cardiovascular disease and aging. Am J Physiol Heart Circ Physiol. 2006 Jun;290(6):H2220-7. Epub 2006 Jan 6. [PubMed:16399855 ]
  9. Wispe JR, Clark JC, Burhans MS, Kropp KE, Korfhagen TR, Whitsett JA: Synthesis and processing of the precursor for human mangano-superoxide dismutase. Biochim Biophys Acta. 1989 Jan 19;994(1):30-6. [PubMed:2462451 ]
  10. Beck Y, Oren R, Amit B, Levanon A, Gorecki M, Hartman JR: Human Mn superoxide dismutase cDNA sequence. Nucleic Acids Res. 1987 Nov 11;15(21):9076. [PubMed:3684581 ]
  11. Heckl K: Isolation of cDNAs encoding human manganese superoxide dismutase. Nucleic Acids Res. 1988 Jul 11;16(13):6224. [PubMed:3399391 ]
  12. Ho YS, Crapo JD: Isolation and characterization of complementary DNAs encoding human manganese-containing superoxide dismutase. FEBS Lett. 1988 Mar 14;229(2):256-60. [PubMed:2831093 ]
  13. Church SL: Manganese superoxide dismutase: nucleotide and deduced amino acid sequence of a cDNA encoding a new human transcript. Biochim Biophys Acta. 1990 Oct 23;1087(2):250-2. [PubMed:1699607 ]
  14. St Clair DK, Holland JC: Complementary DNA encoding human colon cancer manganese superoxide dismutase and the expression of its gene in human cells. Cancer Res. 1991 Feb 1;51(3):939-43. [PubMed:1988135 ]
  15. Wan XS, Devalaraja MN, St Clair DK: Molecular structure and organization of the human manganese superoxide dismutase gene. DNA Cell Biol. 1994 Nov;13(11):1127-36. [PubMed:7702755 ]
  16. Barra D, Schinina ME, Simmaco M, Bannister JV, Bannister WH, Rotilio G, Bossa F: The primary structure of human liver manganese superoxide dismutase. J Biol Chem. 1984 Oct 25;259(20):12595-601. [PubMed:6386798 ]
  17. MacMillan-Crow LA, Thompson JA: Tyrosine modifications and inactivation of active site manganese superoxide dismutase mutant (Y34F) by peroxynitrite. Arch Biochem Biophys. 1999 Jun 1;366(1):82-8. [PubMed:10334867 ]
  18. Borgstahl GE, Parge HE, Hickey MJ, Beyer WF Jr, Hallewell RA, Tainer JA: The structure of human mitochondrial manganese superoxide dismutase reveals a novel tetrameric interface of two 4-helix bundles. Cell. 1992 Oct 2;71(1):107-18. [PubMed:1394426 ]
  19. Borgstahl GE, Parge HE, Hickey MJ, Johnson MJ, Boissinot M, Hallewell RA, Lepock JR, Cabelli DE, Tainer JA: Human mitochondrial manganese superoxide dismutase polymorphic variant Ile58Thr reduces activity by destabilizing the tetrameric interface. Biochemistry. 1996 Apr 9;35(14):4287-97. [PubMed:8605177 ]
  20. Hsieh Y, Guan Y, Tu C, Bratt PJ, Angerhofer A, Lepock JR, Hickey MJ, Tainer JA, Nick HS, Silverman DN: Probing the active site of human manganese superoxide dismutase: the role of glutamine 143. Biochemistry. 1998 Apr 7;37(14):4731-9. [PubMed:9537988 ]
  21. Guan Y, Hickey MJ, Borgstahl GE, Hallewell RA, Lepock JR, O'Connor D, Hsieh Y, Nick HS, Silverman DN, Tainer JA: Crystal structure of Y34F mutant human mitochondrial manganese superoxide dismutase and the functional role of tyrosine 34. Biochemistry. 1998 Apr 7;37(14):4722-30. [PubMed:9537987 ]
  22. Leveque VJ, Stroupe ME, Lepock JR, Cabelli DE, Tainer JA, Nick HS, Silverman DN: Multiple replacements of glutamine 143 in human manganese superoxide dismutase: effects on structure, stability, and catalysis. Biochemistry. 2000 Jun 20;39(24):7131-7. [PubMed:10852710 ]
  23. Hearn AS, Stroupe ME, Cabelli DE, Lepock JR, Tainer JA, Nick HS, Silverman DN: Kinetic analysis of product inhibition in human manganese superoxide dismutase. Biochemistry. 2001 Oct 9;40(40):12051-8. [PubMed:11580280 ]
  24. Perry JJ, Hearn AS, Cabelli DE, Nick HS, Tainer JA, Silverman DN: Contribution of human manganese superoxide dismutase tyrosine 34 to structure and catalysis. Biochemistry. 2009 Apr 21;48(15):3417-24. doi: 10.1021/bi8023288. [PubMed:19265433 ]
  25. Nomiyama T, Tanaka Y, Piao L, Nagasaka K, Sakai K, Ogihara T, Nakajima K, Watada H, Kawamori R: The polymorphism of manganese superoxide dismutase is associated with diabetic nephropathy in Japanese type 2 diabetic patients. J Hum Genet. 2003;48(3):138-41. [PubMed:12624725 ]