Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Sulfite oxidase, mitochondrial

Gene

SUOX

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Catalytic activityi

Sulfite + O2 + H2O = sulfate + H2O2.

Cofactori

Protein has several cofactor binding sites:

Pathwayi: sulfur metabolism

This protein is involved in the pathway sulfur metabolism, which is part of Energy metabolism.
View all proteins of this organism that are known to be involved in the pathway sulfur metabolism and in Energy metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi118Iron (heme axial ligand)1
Metal bindingi143Iron (heme axial ligand)1
Binding sitei145Heme b1 Publication1
Binding sitei147Heme b1 Publication1
Metal bindingi264MolybdenumBy similarity1
Binding sitei322MolybdopterinBy similarity1
Binding sitei361MolybdopterinBy similarity1
Binding sitei366MolybdopterinBy similarity1

GO - Molecular functioni

  • heme binding Source: GO_Central
  • molybdenum ion binding Source: InterPro
  • molybdopterin cofactor binding Source: GO_Central
  • sulfite oxidase activity Source: GO_Central

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
LigandHeme, Iron, Metal-binding, Molybdenum

Enzyme and pathway databases

BioCyciMetaCyc:HS06627-MONOMER
BRENDAi1.8.3.1 2681
ReactomeiR-HSA-1614517 Sulfide oxidation to sulfate
SABIO-RKiP51687
UniPathwayi
UPA00096

Names & Taxonomyi

Protein namesi
Recommended name:
Sulfite oxidase, mitochondrial (EC:1.8.3.1)
Gene namesi
Name:SUOX
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

EuPathDBiHostDB:ENSG00000139531.12
HGNCiHGNC:11460 SUOX
MIMi606887 gene
neXtProtiNX_P51687

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Sulfite oxidase deficiency, isolated (ISOD)5 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA 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.
See also OMIM:272300
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_002200217R → Q in ISOD; 2% of activity. 3 PublicationsCorresponds to variant dbSNP:rs121908007EnsemblClinVar.1
Natural variantiVAR_015724258I → L in ISOD. 1 Publication1
Natural variantiVAR_002201265A → D in ISOD. 2 PublicationsCorresponds to variant dbSNP:rs121908008EnsemblClinVar.1
Natural variantiVAR_015725268R → Q in ISOD. 1 PublicationCorresponds to variant dbSNP:rs1041681662Ensembl.1
Natural variantiVAR_015726362G → S in ISOD. 1 PublicationCorresponds to variant dbSNP:rs757559168Ensembl.1
Natural variantiVAR_015727366R → H in ISOD. 1 PublicationCorresponds to variant dbSNP:rs776690106Ensembl.1
Natural variantiVAR_015728379K → R in ISOD. 1 PublicationCorresponds to variant dbSNP:rs777114729Ensembl.1
Natural variantiVAR_015729396Q → R in ISOD. 1 Publication1
Natural variantiVAR_002202427S → Y in ISOD. 2 Publications1
Natural variantiVAR_015730450W → R in ISOD. 1 Publication1
Natural variantiVAR_002203530G → D in ISOD. 1 PublicationCorresponds to variant dbSNP:rs121908009EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Epilepsy, Mental retardation

Organism-specific databases

DisGeNETi6821
MalaCardsiSUOX
MIMi272300 phenotype
OpenTargetsiENSG00000139531
Orphaneti99731 Isolated sulfite oxidase deficiency
PharmGKBiPA36250

Polymorphism and mutation databases

BioMutaiSUOX
DMDMi152031695

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 79MitochondrionBy similarityAdd BLAST79
ChainiPRO_000000648180 – 545Sulfite oxidase, mitochondrialAdd BLAST466

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei123PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP51687
MaxQBiP51687
PaxDbiP51687
PeptideAtlasiP51687
PRIDEiP51687
ProteomicsDBi56372

PTM databases

iPTMnetiP51687
PhosphoSitePlusiP51687

Expressioni

Gene expression databases

BgeeiENSG00000139531 Expressed in 206 organ(s), highest expression level in right lobe of liver
CleanExiHS_SUOX
ExpressionAtlasiP51687 baseline and differential
GenevisibleiP51687 HS

Organism-specific databases

HPAiHPA038208
HPA038209

Interactioni

Subunit structurei

Homodimer.By similarity

Binary interactionsi

WithEntry#Exp.IntActNotes
NUP62P371985EBI-3921347,EBI-347978

Protein-protein interaction databases

BioGridi112690, 10 interactors
IntActiP51687, 77 interactors
STRINGi9606.ENSP00000266971

Structurei

Secondary structure

1545
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP51687
SMRiP51687
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP51687

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini82 – 161Cytochrome b5 heme-bindingPROSITE-ProRule annotationAdd BLAST80

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni165 – 174HingeBy similarity10
Regioni175 – 401Moco domainBy similarityAdd BLAST227
Regioni215 – 219Molybdopterin-bindingBy similarity5
Regioni377 – 379Molybdopterin-bindingBy similarity3
Regioni402 – 538HomodimerizationBy similarityAdd BLAST137

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0535 Eukaryota
KOG4576 Eukaryota
COG2041 LUCA
GeneTreeiENSGT00390000003749
HOGENOMiHOG000252609
HOVERGENiHBG017865
InParanoidiP51687
KOiK00387
OMAiAVGNAKW
OrthoDBiEOG091G0DBF
PhylomeDBiP51687
TreeFamiTF300905

Family and domain databases

Gene3Di3.10.120.10, 1 hit
3.90.420.10, 1 hit
InterProiView protein in InterPro
IPR001199 Cyt_B5-like_heme/steroid-bd
IPR036400 Cyt_B5-like_heme/steroid_sf
IPR018506 Cyt_B5_heme-BS
IPR014756 Ig_E-set
IPR005066 MoCF_OxRdtse_dimer
IPR008335 Mopterin_OxRdtase_euk
IPR000572 OxRdtase_Mopterin-bd_dom
IPR036374 OxRdtase_Mopterin-bd_sf
IPR022407 OxRdtase_Mopterin_BS
PfamiView protein in Pfam
PF00173 Cyt-b5, 1 hit
PF03404 Mo-co_dimer, 1 hit
PF00174 Oxidored_molyb, 1 hit
PRINTSiPR00363 CYTOCHROMEB5
PR00407 EUMOPTERIN
SMARTiView protein in SMART
SM01117 Cyt-b5, 1 hit
SUPFAMiSSF55856 SSF55856, 1 hit
SSF56524 SSF56524, 1 hit
SSF81296 SSF81296, 1 hit
PROSITEiView protein in PROSITE
PS00191 CYTOCHROME_B5_1, 1 hit
PS50255 CYTOCHROME_B5_2, 1 hit
PS00559 MOLYBDOPTERIN_EUK, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 4 potential isoforms that are computationally mapped.Show allAlign All

P51687-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MLLLHRAVVL RLQQACRLKS IPSRICIQAC STNDSFQPQR PSLTFSGDNS
60 70 80 90 100
STQGWRVMGT LLGLGAVLAY QDHRCRAAQE STHIYTKEEV SSHTSPETGI
110 120 130 140 150
WVTLGSEVFD VTEFVDLHPG GPSKLMLAAG GPLEPFWALY AVHNQSHVRE
160 170 180 190 200
LLAQYKIGEL NPEDKVAPTV ETSDPYADDP VRHPALKVNS QRPFNAEPPP
210 220 230 240 250
ELLTENYITP NPIFFTRNHL PVPNLDPDTY RLHVVGAPGG QSLSLSLDDL
260 270 280 290 300
HNFPRYEITV TLQCAGNRRS EMTQVKEVKG LEWRTGAIST ARWAGARLCD
310 320 330 340 350
VLAQAGHQLC ETEAHVCFEG LDSDPTGTAY GASIPLARAM DPEAEVLLAY
360 370 380 390 400
EMNGQPLPRD HGFPVRVVVP GVVGARHVKW LGRVSVQPEE SYSHWQRRDY
410 420 430 440 450
KGFSPSVDWE TVDFDSAPSI QELPVQSAIT EPRDGETVES GEVTIKGYAW
460 470 480 490 500
SGGGRAVIRV DVSLDGGLTW QVAKLDGEEQ RPRKAWAWRL WQLKAPVPAG
510 520 530 540
QKELNIVCKA VDDGYNVQPD TVAPIWNLRG VLSNAWHRVH VYVSP
Length:545
Mass (Da):60,283
Last modified:July 10, 2007 - v2
Checksum:i39B842C55D39E11F
GO

Computationally mapped potential isoform sequencesi

There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
F8VVW9F8VVW9_HUMAN
Sulfite oxidase, mitochondrial
SUOX
161Annotation score:
F8VPA2F8VPA2_HUMAN
Sulfite oxidase, mitochondrial
SUOX
121Annotation score:
F8VX56F8VX56_HUMAN
Sulfite oxidase, mitochondrial
SUOX
104Annotation score:
F8VRK9F8VRK9_HUMAN
Sulfite oxidase, mitochondrial
SUOX
42Annotation score:

Sequence cautioni

The sequence AAA74886 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAL08048 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_002200217R → Q in ISOD; 2% of activity. 3 PublicationsCorresponds to variant dbSNP:rs121908007EnsemblClinVar.1
Natural variantiVAR_015724258I → L in ISOD. 1 Publication1
Natural variantiVAR_002201265A → D in ISOD. 2 PublicationsCorresponds to variant dbSNP:rs121908008EnsemblClinVar.1
Natural variantiVAR_015725268R → Q in ISOD. 1 PublicationCorresponds to variant dbSNP:rs1041681662Ensembl.1
Natural variantiVAR_015726362G → S in ISOD. 1 PublicationCorresponds to variant dbSNP:rs757559168Ensembl.1
Natural variantiVAR_015727366R → H in ISOD. 1 PublicationCorresponds to variant dbSNP:rs776690106Ensembl.1
Natural variantiVAR_015728379K → R in ISOD. 1 PublicationCorresponds to variant dbSNP:rs777114729Ensembl.1
Natural variantiVAR_015729396Q → R in ISOD. 1 Publication1
Natural variantiVAR_002202427S → Y in ISOD. 2 Publications1
Natural variantiVAR_015730450W → R in ISOD. 1 Publication1
Natural variantiVAR_002203530G → D in ISOD. 1 PublicationCorresponds to variant dbSNP:rs121908009EnsemblClinVar.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L31573 mRNA Translation: AAA74886.1 Different initiation.
BC065193 mRNA Translation: AAH65193.2
AY056018 Genomic DNA Translation: AAL08048.1 Different initiation.
CCDSiCCDS8901.2
PIRiS55874
RefSeqiNP_000447.2, NM_000456.2
NP_001027558.1, NM_001032386.1
NP_001027559.1, NM_001032387.1
XP_016875396.1, XM_017019907.1
XP_016875397.1, XM_017019908.1
UniGeneiHs.558403

Genome annotation databases

EnsembliENST00000266971; ENSP00000266971; ENSG00000139531
ENST00000356124; ENSP00000348440; ENSG00000139531
ENST00000394109; ENSP00000377668; ENSG00000139531
ENST00000394115; ENSP00000377674; ENSG00000139531
ENST00000548274; ENSP00000450245; ENSG00000139531
ENST00000550065; ENSP00000450264; ENSG00000139531
GeneIDi6821
KEGGihsa:6821
UCSCiuc001six.4 human

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L31573 mRNA Translation: AAA74886.1 Different initiation.
BC065193 mRNA Translation: AAH65193.2
AY056018 Genomic DNA Translation: AAL08048.1 Different initiation.
CCDSiCCDS8901.2
PIRiS55874
RefSeqiNP_000447.2, NM_000456.2
NP_001027558.1, NM_001032386.1
NP_001027559.1, NM_001032387.1
XP_016875396.1, XM_017019907.1
XP_016875397.1, XM_017019908.1
UniGeneiHs.558403

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1MJ4X-ray1.20A79-160[»]
ProteinModelPortaliP51687
SMRiP51687
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi112690, 10 interactors
IntActiP51687, 77 interactors
STRINGi9606.ENSP00000266971

PTM databases

iPTMnetiP51687
PhosphoSitePlusiP51687

Polymorphism and mutation databases

BioMutaiSUOX
DMDMi152031695

Proteomic databases

EPDiP51687
MaxQBiP51687
PaxDbiP51687
PeptideAtlasiP51687
PRIDEiP51687
ProteomicsDBi56372

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000266971; ENSP00000266971; ENSG00000139531
ENST00000356124; ENSP00000348440; ENSG00000139531
ENST00000394109; ENSP00000377668; ENSG00000139531
ENST00000394115; ENSP00000377674; ENSG00000139531
ENST00000548274; ENSP00000450245; ENSG00000139531
ENST00000550065; ENSP00000450264; ENSG00000139531
GeneIDi6821
KEGGihsa:6821
UCSCiuc001six.4 human

Organism-specific databases

CTDi6821
DisGeNETi6821
EuPathDBiHostDB:ENSG00000139531.12
GeneCardsiSUOX
HGNCiHGNC:11460 SUOX
HPAiHPA038208
HPA038209
MalaCardsiSUOX
MIMi272300 phenotype
606887 gene
neXtProtiNX_P51687
OpenTargetsiENSG00000139531
Orphaneti99731 Isolated sulfite oxidase deficiency
PharmGKBiPA36250
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0535 Eukaryota
KOG4576 Eukaryota
COG2041 LUCA
GeneTreeiENSGT00390000003749
HOGENOMiHOG000252609
HOVERGENiHBG017865
InParanoidiP51687
KOiK00387
OMAiAVGNAKW
OrthoDBiEOG091G0DBF
PhylomeDBiP51687
TreeFamiTF300905

Enzyme and pathway databases

UniPathwayi
UPA00096

BioCyciMetaCyc:HS06627-MONOMER
BRENDAi1.8.3.1 2681
ReactomeiR-HSA-1614517 Sulfide oxidation to sulfate
SABIO-RKiP51687

Miscellaneous databases

ChiTaRSiSUOX human
EvolutionaryTraceiP51687
GeneWikiiSulfite_oxidase
GenomeRNAii6821
PROiPR:P51687
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000139531 Expressed in 206 organ(s), highest expression level in right lobe of liver
CleanExiHS_SUOX
ExpressionAtlasiP51687 baseline and differential
GenevisibleiP51687 HS

Family and domain databases

Gene3Di3.10.120.10, 1 hit
3.90.420.10, 1 hit
InterProiView protein in InterPro
IPR001199 Cyt_B5-like_heme/steroid-bd
IPR036400 Cyt_B5-like_heme/steroid_sf
IPR018506 Cyt_B5_heme-BS
IPR014756 Ig_E-set
IPR005066 MoCF_OxRdtse_dimer
IPR008335 Mopterin_OxRdtase_euk
IPR000572 OxRdtase_Mopterin-bd_dom
IPR036374 OxRdtase_Mopterin-bd_sf
IPR022407 OxRdtase_Mopterin_BS
PfamiView protein in Pfam
PF00173 Cyt-b5, 1 hit
PF03404 Mo-co_dimer, 1 hit
PF00174 Oxidored_molyb, 1 hit
PRINTSiPR00363 CYTOCHROMEB5
PR00407 EUMOPTERIN
SMARTiView protein in SMART
SM01117 Cyt-b5, 1 hit
SUPFAMiSSF55856 SSF55856, 1 hit
SSF56524 SSF56524, 1 hit
SSF81296 SSF81296, 1 hit
PROSITEiView protein in PROSITE
PS00191 CYTOCHROME_B5_1, 1 hit
PS50255 CYTOCHROME_B5_2, 1 hit
PS00559 MOLYBDOPTERIN_EUK, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiSUOX_HUMAN
AccessioniPrimary (citable) accession number: P51687
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: July 10, 2007
Last modified: November 7, 2018
This is version 191 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  3. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  4. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. Human chromosome 12
    Human chromosome 12: entries, gene names and cross-references to MIM
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again