Entity Details

Primary name SUOX_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP51687
EntryNameSUOX_HUMAN
FullNameSulfite oxidase, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length545
SequenceStatuscomplete
DateCreated1996-10-01
DateModified2021-06-02

Ontological Relatives

GenesSUOX

GO terms

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GOName
GO:0005739 mitochondrion
GO:0005758 mitochondrial intermembrane space
GO:0005759 mitochondrial matrix
GO:0006790 sulfur compound metabolic process
GO:0008482 sulfite oxidase activity
GO:0020037 heme binding
GO:0030151 molybdenum ion binding
GO:0043546 molybdopterin cofactor binding
GO:0070221 sulfide oxidation, using sulfide:quinone oxidoreductase

Subcellular Location

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Subcellular Location
Mitochondrion intermembrane space

Domains

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DomainNameCategoryType
IPR000572 Oxidoreductase, molybdopterin-binding domainDomainDomain
IPR001199 Cytochrome b5-like heme/steroid binding domainDomainDomain
IPR005066 Moybdenum cofactor oxidoreductase, dimerisationDomainDomain
IPR008335 Eukaryotic molybdopterin oxidoreductaseFamilyFamily
IPR014756 Immunoglobulin E-setFamilyHomologous superfamily
IPR018506 Cytochrome b5, heme-binding siteSiteBinding site
IPR022407 Oxidoreductase, molybdopterin binding siteSiteBinding site
IPR036374 Oxidoreductase, molybdopterin-binding domain superfamilyFamilyHomologous superfamily
IPR036400 Cytochrome b5-like heme/steroid binding domain superfamilyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
272300 OMIMSulfite oxidase deficiency, isolated (ISOD)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. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB03983 {[2-Amino-4-oxo-6,7-di(sulfanyl-?S)-3,5,5a,8,9a,10-hexahydro-4H-pyrano[3,2-g]pteridin-8-yl]methyl dihydrogenato(2-) phosphate}(dioxo)sulfanylmolybdenumDrugbanksmall molecule

Interactions

83 interactions

InteractorPartnerSourcesPublicationsLink
SUOX_HUMANZN576_HUMANBioGRID, IntAct21988832 details
SUOX_HUMANECHB_HUMANBioGRID, IntAct21988832 details
SUOX_HUMANK1C27_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANRINT1_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANREL_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANCRX_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANNUP62_HUMANBioGRID, IntAct25910212 32296183 details
SUOX_HUMANCEP76_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANCTG1B_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANGG6L9_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANKRT34_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANBAG4_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANINCA1_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANSGTA_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANKCC2A_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANRCN1_HUMANIntAct32296183 details
SUOX_HUMANPIHD2_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANFANCG_HUMANIntAct32296183 details
SUOX_HUMANZFP90_HUMANIntAct32296183 details
SUOX_HUMANTRI74_HUMANIntAct32296183 details
SUOX_HUMANZN710_HUMANIntAct32296183 details
SUOX_HUMANRHOH_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANDDT4L_HUMANIntAct32296183 details
SUOX_HUMANSTN1_HUMANIntAct32296183 details
SUOX_HUMANMAGBA_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANSYGP1_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANSNF8_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANEYA2_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANTRIB3_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANCDCA4_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANMORN3_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANK1C26_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANPRRX1_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANMYO1B_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANMB3L1_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANNB5R2_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANPTTG2_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANACO11_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANDUS21_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANEIF3F_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANKS6R_HUMANIntAct32296183 details
SUOX_HUMANCOA7_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANMLRA_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANMED28_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANF217B_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANSPC24_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANZXDC_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANVPS28_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANMEIS3_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANCSN8_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANC1D_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANTEX12_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANCENPR_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANUBR2_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANMDFI_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANSRTD3_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANMOONR_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANMAGAB_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANK1C40_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANUBQL2_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANISCA2_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANDMAP1_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANPEX19_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANCOLI_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANRUSC1_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANCC103_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANZN143_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANSOLH1_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANLMO4_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANHM20A_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANSTH_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANSTA5B_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANERIC4_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANPFD3_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANFND3B_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANRLA1_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANING5_HUMANBioGRID, IntAct32296183 details
SUOX_HUMAN3BP5L_HUMANBioGRID, IntAct32296183 details
SUOX_HUMANFMR1_HUMANBioGRID32296183 details
SUOX_HUMANFMNL2_HUMANBioGRID32296183 details
SUOX_HUMANTSC1_HUMANBioGRID32296183 details
SUOX_HUMANTF65_HUMANBioGRID32296183 details
SUOX_HUMANCE051_HUMANBioGRID32296183 details