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Protein

Sulfite oxidase, mitochondrial

Gene

SUOX

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Ligandi

Heme, Iron, Metal-binding, Molybdenum

Enzyme and pathway databases

BioCyciMetaCyc:HS06627-MONOMER.
ZFISH:HS06627-MONOMER.
BRENDAi1.8.3.1. 2681.
ReactomeiR-HSA-1614517. Sulfide oxidation to sulfate.
SABIO-RKP51687.
UniPathwayiUPA00096.

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

HGNCiHGNC:11460. SUOX.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Isolated sulfite oxidase deficiency (ISOD)5 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionCharacterized by neurological abnormalities including multicystic leukoencephalopathy with brain atrophy. Patients often suffer from seizures. Often leads to death at an early age.
See also OMIM:272300
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_002200217R → Q in ISOD; 2% of activity. 3 PublicationsCorresponds to variant rs121908007dbSNPEnsembl.1
Natural variantiVAR_015724258I → L in ISOD. 1 Publication1
Natural variantiVAR_002201265A → D in ISOD. 2 PublicationsCorresponds to variant rs121908008dbSNPEnsembl.1
Natural variantiVAR_015725268R → Q in ISOD. 1 Publication1
Natural variantiVAR_015726362G → S in ISOD. 1 PublicationCorresponds to variant rs757559168dbSNPEnsembl.1
Natural variantiVAR_015727366R → H in ISOD. 1 PublicationCorresponds to variant rs776690106dbSNPEnsembl.1
Natural variantiVAR_015728379K → R in ISOD. 1 PublicationCorresponds to variant rs777114729dbSNPEnsembl.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 rs121908009dbSNPEnsembl.1

Keywords - Diseasei

Disease mutation

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.

PTM databases

iPTMnetiP51687.
PhosphoSitePlusiP51687.

Expressioni

Gene expression databases

BgeeiENSG00000139531.
CleanExiHS_SUOX.
ExpressionAtlasiP51687. baseline and differential.
GenevisibleiP51687. HS.

Organism-specific databases

HPAiHPA038208.
HPA038209.

Interactioni

Subunit structurei

Homodimer.By similarity

Protein-protein interaction databases

BioGridi112690. 8 interactors.
IntActiP51687. 19 interactors.
STRINGi9606.ENSP00000266971.

Structurei

Secondary structure

1545
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Turni96 – 98Combined sources3
Beta strandi99 – 104Combined sources6
Beta strandi107 – 110Combined sources4
Turni112 – 114Combined sources3
Helixi115 – 117Combined sources3
Helixi122 – 126Combined sources5
Turni127 – 130Combined sources4
Beta strandi131 – 133Combined sources3
Helixi134 – 137Combined sources4
Helixi141 – 143Combined sources3
Helixi146 – 153Combined sources8
Beta strandi156 – 159Combined sources4

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...

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

Sequence similaritiesi

Contains 1 cytochrome b5 heme-binding domain.PROSITE-ProRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

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

Family and domain databases

Gene3Di2.60.40.650. 1 hit.
3.10.120.10. 1 hit.
3.90.420.10. 1 hit.
InterProiIPR001199. Cyt_B5-like_heme/steroid-bd.
IPR018506. Cyt_B5_heme-BS.
IPR014756. Ig_E-set.
IPR005066. MoCF_OxRdtse_dimer.
IPR008335. Mopterin_OxRdtase_euk.
IPR000572. OxRdtase_Mopterin-bd_dom.
IPR022407. OxRdtase_Mopterin_BS.
[Graphical view]
PfamiPF00173. Cyt-b5. 1 hit.
PF03404. Mo-co_dimer. 1 hit.
PF00174. Oxidored_molyb. 1 hit.
[Graphical view]
PRINTSiPR00363. CYTOCHROMEB5.
PR00407. EUMOPTERIN.
SMARTiSM01117. Cyt-b5. 1 hit.
[Graphical view]
SUPFAMiSSF55856. SSF55856. 1 hit.
SSF56524. SSF56524. 1 hit.
SSF81296. SSF81296. 1 hit.
PROSITEiPS00191. CYTOCHROME_B5_1. 1 hit.
PS50255. CYTOCHROME_B5_2. 1 hit.
PS00559. MOLYBDOPTERIN_EUK. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

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

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 rs121908007dbSNPEnsembl.1
Natural variantiVAR_015724258I → L in ISOD. 1 Publication1
Natural variantiVAR_002201265A → D in ISOD. 2 PublicationsCorresponds to variant rs121908008dbSNPEnsembl.1
Natural variantiVAR_015725268R → Q in ISOD. 1 Publication1
Natural variantiVAR_015726362G → S in ISOD. 1 PublicationCorresponds to variant rs757559168dbSNPEnsembl.1
Natural variantiVAR_015727366R → H in ISOD. 1 PublicationCorresponds to variant rs776690106dbSNPEnsembl.1
Natural variantiVAR_015728379K → R in ISOD. 1 PublicationCorresponds to variant rs777114729dbSNPEnsembl.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 rs121908009dbSNPEnsembl.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.

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. 8 interactors.
IntActiP51687. 19 interactors.
STRINGi9606.ENSP00000266971.

PTM databases

iPTMnetiP51687.
PhosphoSitePlusiP51687.

Polymorphism and mutation databases

BioMutaiSUOX.
DMDMi152031695.

Proteomic databases

EPDiP51687.
MaxQBiP51687.
PaxDbiP51687.
PeptideAtlasiP51687.
PRIDEiP51687.

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.
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.
OMAiAVHNQSH.
OrthoDBiEOG091G0DBF.
PhylomeDBiP51687.
TreeFamiTF300905.

Enzyme and pathway databases

UniPathwayiUPA00096.
BioCyciMetaCyc:HS06627-MONOMER.
ZFISH:HS06627-MONOMER.
BRENDAi1.8.3.1. 2681.
ReactomeiR-HSA-1614517. Sulfide oxidation to sulfate.
SABIO-RKP51687.

Miscellaneous databases

ChiTaRSiSUOX. human.
EvolutionaryTraceiP51687.
GeneWikiiSulfite_oxidase.
GenomeRNAii6821.
PROiP51687.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000139531.
CleanExiHS_SUOX.
ExpressionAtlasiP51687. baseline and differential.
GenevisibleiP51687. HS.

Family and domain databases

Gene3Di2.60.40.650. 1 hit.
3.10.120.10. 1 hit.
3.90.420.10. 1 hit.
InterProiIPR001199. Cyt_B5-like_heme/steroid-bd.
IPR018506. Cyt_B5_heme-BS.
IPR014756. Ig_E-set.
IPR005066. MoCF_OxRdtse_dimer.
IPR008335. Mopterin_OxRdtase_euk.
IPR000572. OxRdtase_Mopterin-bd_dom.
IPR022407. OxRdtase_Mopterin_BS.
[Graphical view]
PfamiPF00173. Cyt-b5. 1 hit.
PF03404. Mo-co_dimer. 1 hit.
PF00174. Oxidored_molyb. 1 hit.
[Graphical view]
PRINTSiPR00363. CYTOCHROMEB5.
PR00407. EUMOPTERIN.
SMARTiSM01117. Cyt-b5. 1 hit.
[Graphical view]
SUPFAMiSSF55856. SSF55856. 1 hit.
SSF56524. SSF56524. 1 hit.
SSF81296. SSF81296. 1 hit.
PROSITEiPS00191. CYTOCHROME_B5_1. 1 hit.
PS50255. CYTOCHROME_B5_2. 1 hit.
PS00559. MOLYBDOPTERIN_EUK. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiSUOX_HUMAN
AccessioniPrimary (citable) accession number: P51687
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: July 10, 2007
Last modified: November 30, 2016
This is version 178 of the entry and version 2 of the sequence. [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 chromosome 12
    Human chromosome 12: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.