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Identification
HMDB Protein ID HMDBP01057
Secondary Accession Numbers
  • 6346
Name Sulfite oxidase, mitochondrial
Synonyms Not Available
Gene Name SUOX
Protein Type Unknown
Biological Properties
General Function Involved in heme binding
Specific Function Not Available
Pathways
  • Sulfate/Sulfite Metabolism
  • Sulfite oxidase deficiency
  • sulfur metabolism
Reactions
Sulfite + Oxygen + Water → Oat gum + Hydrogen peroxide details
GO Classification
Biological Process
cellular nitrogen compound metabolic process
sulfur amino acid catabolic process
response to nutrient
sulfide oxidation, using sulfide:quinone oxidoreductase
Cellular Component
cytosol
mitochondrial matrix
mitochondrial intermembrane space
Function
ion binding
cation binding
metal ion binding
binding
catalytic activity
transition metal ion binding
electron carrier activity
iron ion binding
heme binding
molybdenum ion binding
oxidoreductase activity
Molecular Function
electron carrier activity
molybdenum ion binding
molybdopterin cofactor binding
sulfite oxidase activity
heme binding
Process
metabolic process
oxidation reduction
Cellular Location
  1. Mitochondrion intermembrane space
Gene Properties
Chromosome Location 12
Locus 12q13.2
SNPs SUOX
Gene Sequence
>1638 bp
ATGCTGCTGCTGCACAGAGCTGTGGTCCTCAGGCTCCAACAGGCCTGCAGACTCAAGTCA
ATCCCCTCAAGGATCTGCATTCAGGCCTGCTCCACAAATGATTCATTTCAGCCCCAGCGC
CCCAGCCTCACCTTCTCTGGTGATAACTCCAGCACCCAGGGATGGAGAGTCATGGGGACC
CTATTAGGTCTCGGTGCAGTGTTGGCCTATCAGGACCATCGGTGTAGGGCTGCTCAGGAG
TCAACACACATATACACTAAGGAGGAAGTGAGTTCCCACACCAGCCCTGAGACTGGGATC
TGGGTGACTCTGGGCTCTGAGGTCTTTGATGTCACAGAATTTGTGGACCTACATCCAGGG
GGGCCTTCAAAGCTGATGCTAGCAGCTGGGGGTCCCCTAGAGCCCTTCTGGGCCCTCTAT
GCTGTTCACAACCAGTCCCATGTGCGTGAGTTACTGGCTCAGTACAAGATTGGGGAGCTG
AATCCTGAAGACAAGGTAGCCCCCACCGTGGAGACCTCTGACCCTTATGCTGATGATCCT
GTACGTCACCCAGCCCTGAAGGTCAACAGCCAGCGGCCCTTTAATGCAGAGCCTCCCCCT
GAGCTGCTGACAGAAAACTACATCACACCCAACCCTATCTTCTTCACCCGGAACCATCTG
CCTGTACCTAACCTGGATCCAGACACCTATCGCTTACACGTAGTAGGAGCACCTGGGGGT
CAGTCACTGTCTCTTTCCCTGGATGACTTGCACAACTTTCCCAGGTACGAGATCACAGTC
ACTCTGCAGTGTGCCGGCAACCGACGCTCTGAGATGACTCAGGTCAAAGAAGTAAAAGGT
CTGGAGTGGAGAACAGGAGCCATCAGCACTGCACGCTGGGCTGGGGCACGGCTCTGTGAT
GTGTTAGCCCAGGCTGGCCACCAACTCTGTGAAACTGAGGCCCACGTCTGCTTTGAGGGA
CTGGACTCAGACCCTACTGGGACTGCCTATGGAGCATCCATCCCTCTGGCTCGGGCCATG
GACCCTGAAGCTGAGGTCCTGCTGGCATATGAGATGAATGGGCAGCCTCTGCCACGTGAC
CACGGCTTCCCTGTGCGTGTGGTGGTTCCTGGAGTGGTGGGTGCCCGCCATGTCAAATGG
CTGGGCAGAGTGAGTGTGCAGCCAGAGGAAAGTTACAGCCACTGGCAACGGCGGGATTAC
AAAGGCTTCTCTCCATCTGTGGACTGGGAGACTGTAGATTTTGACTCTGCTCCATCCATT
CAGGAACTTCCTGTCCAGTCGGCCATCACAGAGCCCCGGGATGGAGAGACTGTAGAATCA
GGGGAGGTGACCATCAAGGGCTATGCATGGAGTGGTGGTGGCAGGGCTGTGATCCGGGTG
GATGTGTCTCTGGATGGGGGCCTAACCTGGCAGGTGGCTAAGCTGGATGGAGAGGAACAG
CGCCCCAGGAAGGCCTGGGCATGGCGTCTGTGGCAGTTGAAAGCCCCTGTGCCAGCTGGA
CAAAAGGAACTGAACATTGTTTGTAAGGCTGTGGATGATGGTTACAATGTGCAGCCAGAC
ACCGTGGCCCCAATCTGGAACCTGCGAGGTGTTCTCAGCAATGCCTGGCATCGTGTCCAT
GTCTATGTCTCCCCATGA
Protein Properties
Number of Residues 545
Molecular Weight 60282.59
Theoretical pI 6.107
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Sulfite oxidase, mitochondrial
MLLLHRAVVLRLQQACRLKSIPSRICIQACSTNDSFQPQRPSLTFSGDNSSTQGWRVMGT
LLGLGAVLAYQDHRCRAAQESTHIYTKEEVSSHTSPETGIWVTLGSEVFDVTEFVDLHPG
GPSKLMLAAGGPLEPFWALYAVHNQSHVRELLAQYKIGELNPEDKVAPTVETSDPYADDP
VRHPALKVNSQRPFNAEPPPELLTENYITPNPIFFTRNHLPVPNLDPDTYRLHVVGAPGG
QSLSLSLDDLHNFPRYEITVTLQCAGNRRSEMTQVKEVKGLEWRTGAISTARWAGARLCD
VLAQAGHQLCETEAHVCFEGLDSDPTGTAYGASIPLARAMDPEAEVLLAYEMNGQPLPRD
HGFPVRVVVPGVVGARHVKWLGRVSVQPEESYSHWQRRDYKGFSPSVDWETVDFDSAPSI
QELPVQSAITEPRDGETVESGEVTIKGYAWSGGGRAVIRVDVSLDGGLTWQVAKLDGEEQ
RPRKAWAWRLWQLKAPVPAGQKELNIVCKAVDDGYNVQPDTVAPIWNLRGVLSNAWHRVH
VYVSP
GenBank ID Protein 74099694
UniProtKB/Swiss-Prot ID P51687
UniProtKB/Swiss-Prot Entry Name SUOX_HUMAN
PDB IDs
GenBank Gene ID NM_000456.2
GeneCard ID SUOX
GenAtlas ID SUOX
HGNC ID HGNC:11460
References
General References
  1. 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 ]
  2. Garrett RM, Bellissimo DB, Rajagopalan KV: Molecular cloning of human liver sulfite oxidase. Biochim Biophys Acta. 1995 Jun 9;1262(2-3):147-9. [PubMed:7599189 ]
  3. Rudolph MJ, Johnson JL, Rajagopalan KV, Kisker C: The 1.2 A structure of the human sulfite oxidase cytochrome b(5) domain. Acta Crystallogr D Biol Crystallogr. 2003 Jul;59(Pt 7):1183-91. Epub 2003 Jun 27. [PubMed:12832761 ]
  4. Kisker C, Schindelin H, Pacheco A, Wehbi WA, Garrett RM, Rajagopalan KV, Enemark JH, Rees DC: Molecular basis of sulfite oxidase deficiency from the structure of sulfite oxidase. Cell. 1997 Dec 26;91(7):973-83. [PubMed:9428520 ]
  5. Garrett RM, Johnson JL, Graf TN, Feigenbaum A, Rajagopalan KV: Human sulfite oxidase R160Q: identification of the mutation in a sulfite oxidase-deficient patient and expression and characterization of the mutant enzyme. Proc Natl Acad Sci U S A. 1998 May 26;95(11):6394-8. [PubMed:9600976 ]
  6. Edwards MC, Johnson JL, Marriage B, Graf TN, Coyne KE, Rajagopalan KV, MacDonald IM: Isolated sulfite oxidase deficiency: review of two cases in one family. Ophthalmology. 1999 Oct;106(10):1957-61. [PubMed:10519592 ]
  7. Johnson JL, Coyne KE, Garrett RM, Zabot MT, Dorche C, Kisker C, Rajagopalan KV: Isolated sulfite oxidase deficiency: identification of 12 novel SUOX mutations in 10 patients. Hum Mutat. 2002 Jul;20(1):74. [PubMed:12112661 ]
  8. Lee HF, Mak BS, Chi CS, Tsai CR, Chen CH, Shu SG: A novel mutation in neonatal isolated sulphite oxidase deficiency. Neuropediatrics. 2002 Aug;33(4):174-9. [PubMed:12368985 ]