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

More general terms

• oxidoreductase
• mitochondrial protein
• metalloprotein
• oligomerizing protein
• phosphoprotein

Additional terms

• molybdenum cofactor deficiency
• sulfocysteinuria; isolated sulfite oxidase deficiency

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=6821
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:6821
• OMIM: https://mirror.omim.org/entry/272300
• OMIM: https://mirror.omim.org/entry/606887
• UniProt: http://www.uniprot.org/uniprot/P51687.html