SUOX
Sulfite oxidase, mitochondrial
Also known as: SUOX_HUMAN
Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene
Protein identityUniProt · HPA
- UniProt accession
- P51687
- Gene
- SUOX
- Ensembl
- ENSG00000139531
- Chromosome
- 12
- Canonical length
- 545 aa
- Protein class
- Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets, Predicted intracellular proteins
- Quaternary structure
- Homodimer
OverviewNCBI Gene
Sulfite oxidase is a homodimeric protein localized to the intermembrane space of mitochondria. Each subunit contains a heme domain and a molybdopterin-binding domain. The enzyme catalyzes the oxidation of sulfite to sulfate, the final reaction in the oxidative degradation of the sulfur amino acids cysteine and methionine. Sulfite oxidase deficiency results in neurological abnormalities which are often fatal at an early age. Alternative splicing results in multiple transcript variants encoding identical proteins. [provided by RefSeq, Jul 2008]
Canonical amino-acid sequenceUniProt
545 residues, UniProt reviewed canonical sequence.
>P51687|SUOX
1 MLLLHRAVVL RLQQACRLKS IPSRICIQAC STNDSFQPQR PSLTFSGDNS STQGWRVMGT
61 LLGLGAVLAY QDHRCRAAQE STHIYTKEEV SSHTSPETGI WVTLGSEVFD VTEFVDLHPG
121 GPSKLMLAAG GPLEPFWALY AVHNQSHVRE LLAQYKIGEL NPEDKVAPTV ETSDPYADDP
181 VRHPALKVNS QRPFNAEPPP ELLTENYITP NPIFFTRNHL PVPNLDPDTY RLHVVGAPGG
241 QSLSLSLDDL HNFPRYEITV TLQCAGNRRS EMTQVKEVKG LEWRTGAIST ARWAGARLCD
301 VLAQAGHQLC ETEAHVCFEG LDSDPTGTAY GASIPLARAM DPEAEVLLAY EMNGQPLPRD
361 HGFPVRVVVP GVVGARHVKW LGRVSVQPEE SYSHWQRRDY KGFSPSVDWE TVDFDSAPSI
421 QELPVQSAIT EPRDGETVES GEVTIKGYAW SGGGRAVIRV DVSLDGGLTW QVAKLDGEEQ
481 RPRKAWAWRL WQLKAPVPAG QKELNIVCKA VDDGYNVQPD TVAPIWNLRG VLSNAWHRVH
541 VYVSPLocalizationUniProt · AlphaFold · HPA
Whether an antibody against SUOX can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.
- Antibody reachability
- Other membrane
- Secreted
- No
- Transmembrane segments
- 0
- Mean surface accessibility (rSASA)
- 0.3
- Highest tissue expression
- 70 nTPM
Expression across tissuesHPA
Tissue
- liver: 70 nTPM
- parathyroid gland: 46 nTPM
- skeletal muscle: 43 nTPM
- kidney: 38 nTPM
- adrenal gland: 33 nTPM
- epididymis: 31 nTPM
Single-cell type
- hepatocytes: 56 nCPM
- cytotrophoblasts: 45 nCPM
- syncytiotrophoblasts: 42 nCPM
- migrating cytotrophoblasts: 35 nCPM
- esophageal apical cells: 31 nCPM
- adrenal cortex cells: 30 nCPM
Immune cell
- eosinophil: 30 nTPM
- intermediate monocyte: 19 nTPM
- NK-cell: 17 nTPM
- non-classical monocyte: 16 nTPM
- myeloid DC: 16 nTPM
- classical monocyte: 13 nTPM
Brain region
- white matter: 36 nTPM
- choroid plexus: 36 nTPM
- thalamus: 33 nTPM
- cerebellum: 33 nTPM
- cerebral cortex: 32 nTPM
- pons: 32 nTPM
DiseaseUniProt · ClinVar · IEDB · PubMed
Four sources answering four different questions about SUOX.
Disease | AllUniProt
Conditions SUOX is implicated in, by any mechanism.
- Sulfite oxidase deficiency, isolated (ISOD) MIM:272300
Disease | GeneticClinVar
79 pathogenic / likely-pathogenic of 517 ClinVar records.
Conditions with pathogenic or likely-pathogenic variants.
- Sulfite oxidase deficiency
- Sulfocysteinuria
- SUOX-related disorder
- Inborn genetic diseases
Genetic constraint and essentialitygnomAD · DepMap
Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.
- gnomAD LOEUF (loss-of-function intolerance)
- 1.08
- gnomAD pLI
- 0
- gnomAD missense Z
- 0.45
- DepMap mean gene effect
- -0.25
- DepMap dependency class
- selective
Cancer expressionTCGA
Across TCGA tumor cohorts, this protein is over-expressed in roughly 5% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).
OntologyGO
Biological processes
Molecular functions
- heme binding
- molybdenum ion binding
- molybdopterin cofactor binding
- sulfite oxidase activity
Cellular components
Protein domainsUniProt · Pfam · InterPro
- Cytochrome b5-like heme/steroid binding domain
- Immunoglobulin E-set
- Cytochrome b5, heme-binding site
- Oxidoreductase, molybdopterin binding site
- Cytochrome b5-like heme/steroid binding domain superfamily
- Cytochrome b5-like Heme/Steroid binding domain
- Oxidoreductase, molybdopterin-binding domain
- Moybdenum cofactor oxidoreductase, dimerisation
- Eukaryotic molybdopterin oxidoreductase
- Oxidoreductase, molybdopterin-binding domain superfamily
- Oxidoreductase molybdopterin binding domain
- Mo-co oxidoreductase dimerisation domain
KeywordsUniProt
Antibody and autoantibody relevanceSeroatlas analysis
Seroatlas reads SUOX as an antibody target. Whether an autoantibody or antibody against SUOX could matter depends on whether native SUOX is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.
SUOX is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.
Annotation status
The present source text does not explicitly label SUOX as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.
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