SUOX
entrez
6821
canonical
uniprot
P51687
canonical
ensembl
ENSG00000139531
resolver
ensp
ENSP00000266971
resolver
ensp
ENSP00000348440
resolver
ensp
ENSP00000377668
resolver
ensp
ENSP00000377674
resolver
ensp
ENSP00000450245
resolver
ensp
ENSP00000450264
resolver
ensp
ENSP00000556471
resolver
ensp
ENSP00000556472
resolver
ensp
ENSP00000556473
resolver
ensp
ENSP00000556474
resolver
ensp
ENSP00000556475
resolver
ensp
ENSP00000556476
resolver
ensp
ENSP00000556477
resolver
ensp
ENSP00000556478
resolver
ensp
ENSP00000556479
resolver
ensp
ENSP00000556480
resolver
ensp
ENSP00000556481
resolver
ensp
ENSP00000556482
resolver
ensp
ENSP00000556484
resolver
ensp
ENSP00000556485
resolver
ensp
ENSP00000556486
resolver
ensp
ENSP00000556487
resolver
ensp
ENSP00000556488
resolver
ensp
ENSP00000556489
resolver
ensp
ENSP00000556490
resolver
ensp
ENSP00000556491
resolver
ensp
ENSP00000556492
resolver
ensp
ENSP00000556493
resolver
ensp
ENSP00000556494
resolver
ensp
ENSP00000556495
resolver
ensp
ENSP00000556496
resolver
ensp
ENSP00000556497
resolver
ensp
ENSP00000556498
resolver
ensp
ENSP00000556499
resolver
ensp
ENSP00000600362
resolver
ensp
ENSP00000600363
resolver
ensp
ENSP00000600364
resolver
ensp
ENSP00000600365
resolver
ensp
ENSP00000600366
resolver
ensp
ENSP00000600367
resolver
ensp
ENSP00000624673
resolver
ensp
ENSP00000624675
resolver
ensp
ENSP00000624676
resolver
enst
ENST00000266971
resolver
enst
ENST00000356124
resolver
enst
ENST00000394109
resolver
enst
ENST00000394115
resolver
enst
ENST00000548274
resolver
enst
ENST00000550065
resolver
enst
ENST00000886412
resolver
enst
ENST00000886413
resolver
enst
ENST00000886414
resolver
enst
ENST00000886415
resolver
enst
ENST00000886416
resolver
enst
ENST00000886417
resolver
enst
ENST00000886418
resolver
enst
ENST00000886419
resolver
enst
ENST00000886420
resolver
enst
ENST00000886421
resolver
enst
ENST00000886422
resolver
enst
ENST00000886423
resolver
enst
ENST00000886425
resolver
enst
ENST00000886426
resolver
enst
ENST00000886427
resolver
enst
ENST00000886428
resolver
enst
ENST00000886429
resolver
enst
ENST00000886430
resolver
enst
ENST00000886431
resolver
enst
ENST00000886432
resolver
enst
ENST00000886433
resolver
enst
ENST00000886434
resolver
enst
ENST00000886435
resolver
enst
ENST00000886436
resolver
enst
ENST00000886437
resolver
enst
ENST00000886438
resolver
enst
ENST00000886439
resolver
enst
ENST00000886440
resolver
enst
ENST00000930303
resolver
enst
ENST00000930304
resolver
enst
ENST00000930305
resolver
enst
ENST00000930306
resolver
enst
ENST00000930307
resolver
enst
ENST00000930308
resolver
enst
ENST00000954614
resolver
enst
ENST00000954616
resolver
enst
ENST00000954617
resolver
genesymbol
SUOX
resolver
hgnc
HGNC:11460
resolver
uniprot_entry
SUOX_HUMAN
resolver
amino_acid_sequence
MLLLHRAVVLRLQQACRLKSIPSRICIQACSTNDSFQPQRPSLTFSGDNSSTQGWRVMGTLLGLGAVLAYQDHRCRAAQESTHIYTKEEVSSHTSPETGIWVTLGSEVFDVTEFVDLHPGGPSKLMLAAGGPLEPFWALYAVHNQSHVRELLAQYKIGELNPEDKVAPTVETSDPYADDPVRHPALKVNSQRPFNAEPPPELLTENYITPNPIFFTRNHLPVPNLDPDTYRLHVVGAPGGQSLSLSLDDLHNFPRYEITVTLQCAGNRRSEMTQVKEVKGLEWRTGAISTARWAGARLCDVLAQAGHQLCETEAHVCFEGLDSDPTGTAYGASIPLARAMDPEAEVLLAYEMNGQPLPRDHGFPVRVVVPGVVGARHVKWLGRVSVQPEESYSHWQRRDYKGFSPSVDWETVDFDSAPSIQELPVQSAITEPRDGETVESGEVTIKGYAWSGGGRAVIRVDVSLDGGLTWQVAKLDGEEQRPRKAWAWRLWQLKAPVPAGQKELNIVCKAVDDGYNVQPDTVAPIWNLRGVLSNAWHRVHVYVSP
uniprot
proteins
disease_involvement
DISEASE: Sulfite oxidase deficiency, isolated (ISOD) [MIM:272300]: A life-threatening, autosomal recessive neurometabolic disorder characterized by severe neurological impairment. Classic ISOD manifests in the first few hours to days of life and is characterized by intractable seizures, feeding difficulties, rapidly progressive encephalopathy, microcephaly, and profound intellectual disability. Children usually die during the first few months of life. Mild ISOD manifests in infancy or early childhood and is characterized by ectopia lentis that is variably present, developmental delay and regression, movement disorder characterized by dystonia and choreoathetosis, ataxia, and rarely acute hemiplegia due to metabolic stroke. {ECO:0000269|PubMed:10519592, ECO:0000269|PubMed:12112661, ECO:0000269|PubMed:12368985, ECO:0000269|PubMed:19793632, ECO:0000269|PubMed:27289259, ECO:0000269|PubMed:9428520, ECO:0000269|PubMed:9600976}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
ec_number
1.7.-.-
uniprot
proteins
ec_number
1.8.3.1
uniprot
proteins
function
FUNCTION: Catalyzes the oxidation of sulfite to sulfate, the terminal reaction in the oxidative degradation of sulfur-containing amino acids (By similarity). Can run in reverse direction and reduce nitrite to nitric oxide under physiologically relevant conditions, such as hypoxia (PubMed:41337830). {ECO:0000250|UniProtKB:P07850, ECO:0000269|PubMed:41337830}.
uniprot
proteins
mass_dalton
60283
uniprot
proteins
pathway_participation
PATHWAY: Energy metabolism; sulfur metabolism. {ECO:0000250|UniProtKB:Q07116}.
uniprot
proteins
pubmed
10519592
uniprot
proteins
pubmed
12112661
uniprot
proteins
pubmed
12368985
uniprot
proteins
pubmed
12832761
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
19793632
uniprot
proteins
pubmed
23163752
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
27289259
uniprot
proteins
pubmed
41337830
uniprot
proteins
pubmed
7599189
uniprot
proteins
pubmed
9428520
uniprot
proteins
pubmed
9600976
uniprot
proteins
sequence_length
545
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion intermembrane space {ECO:0000250|UniProtKB:Q07116}.
uniprot
proteins