MGP Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins
MGP003024

UniProt Annotations

Entry Information
Gene Namesulfite oxidase
Protein EntrySUOX_HUMAN
UniProt IDP51687
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivitySulfite + O(2) + H(2)O = sulfate + H(2)O(2).
CofactorName=heme b; Xref=ChEBI:CHEBI:60344; Evidence={ECO:0000269|PubMed:12832761}; Note=Binds 1 heme b (iron(II)-protoporphyrin IX) group non- covalently per subunit. {ECO:0000269|PubMed:12832761};
CofactorName=Mo-molybdopterin; Xref=ChEBI:CHEBI:71302; Evidence={ECO:0000250}; Note=Binds 1 Mo-molybdopterin (Mo-MPT) cofactor per subunit. {ECO:0000250};
DiseaseIsolated sulfite oxidase deficiency (ISOD) [MIM:272300]: Characterized by neurological abnormalities including multicystic leukoencephalopathy with brain atrophy. Patients often suffer from seizures. Often leads to death at an early age. {ECO:0000269|PubMed:10519592, ECO:0000269|PubMed:12112661, ECO:0000269|PubMed:12368985, ECO:0000269|PubMed:9428520, ECO:0000269|PubMed:9600976}. Note=The disease is caused by mutations affecting the gene represented in this entry.
PathwayEnergy metabolism; sulfur metabolism.
Sequence CautionSequence=AAA74886.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence={ECO:0000305}; Sequence=AAL08048.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence={ECO:0000305};
SimilarityContains 1 cytochrome b5 heme-binding domain. {ECO:0000255|PROSITE-ProRule:PRU00279}.
Subcellular LocationMitochondrion intermembrane space.
SubunitHomodimer. {ECO:0000250}.
  logo