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sulfite oxidase, mitochondrial (SUOX)
Function:
- energy metabolism; sulfur metabolism
sulfite + O2 + H2O = sulfate + H2O2
Cofactor:
- binds 1 protoheme group
- molybdenum (molybdopterin)
Structure:
- homodimer (putative)
- contains 1 cytochrome b5 heme-binding domain
Compartment: mitochondria intermembrane space
Pathology:
- defects in SUOX are the cause of sulfocysteinuria
Related
molybdenum cofactor deficiency
sulfocysteinuria; isolated sulfite oxidase deficiency
General
metalloprotein
mitochondrial protein
oligomerizing protein
oxidoreductase
phosphoprotein
Properties
SIZE: entity length = 545 aa
MW = 60 kD
COMPARTMENT: mitochondria
MOTIF: heme-binding site [82-161]
MOTIF: Iron [Fe]-binding site
SITE: 118-118
Iron [Fe]-binding site
SITE: 143-143
Hinge {165-181}
Molybdenum-pterin domain {182-545}
MOTIF: binding site
SITE: 215-219
FOR-BINDING-OF: MOLYBDENUM-PTERIN
molybdenum [Mo]-pterin-binding site
SITE: 264-264
Thr phosphorylation site {T285}
molybdenum [Mo]-pterin-binding site
SITE: 317-317
binding site
SITE: 320-322
FOR-BINDING-OF: MOLYBDENUM-PTERIN
binding site
SITE: 366-379
FOR-BINDING-OF: MOLYBDENUM-PTERIN
Database Correlations
OMIM correlations
MORBIDMAP 606887
UniProt P51687
PFAM correlations
Entrez Gene 6821
Kegg hsa:6821
ENZYME 1.8.3.1
References
- UniProt :accession P51687
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/SUOX