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P51687

- SUOX_HUMAN

UniProt

P51687 - SUOX_HUMAN

Protein

Sulfite oxidase, mitochondrial

Gene

SUOX

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 157 (01 Oct 2014)
      Sequence version 2 (10 Jul 2007)
      Previous versions | rss
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    Functioni

    Catalytic activityi

    Sulfite + O2 + H2O = sulfate + H2O2.

    Cofactori

    Binds 1 heme B (iron-protoporphyrin IX) group non-covalently per subunit.1 Publication
    Binds 1 molybdenum-molybdopterin (Mo-MPT) cofactor per subunit.By similarity

    Pathwayi

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Metal bindingi118 – 1181Iron (heme axial ligand)
    Metal bindingi143 – 1431Iron (heme axial ligand)
    Binding sitei145 – 1451Heme b1 Publication
    Binding sitei147 – 1471Heme b1 Publication
    Metal bindingi264 – 2641MolybdenumBy similarity
    Binding sitei322 – 3221MolybdopterinBy similarity
    Binding sitei361 – 3611MolybdopterinBy similarity
    Binding sitei366 – 3661MolybdopterinBy similarity

    GO - Molecular functioni

    1. electron carrier activity Source: InterPro
    2. heme binding Source: InterPro
    3. molybdenum ion binding Source: InterPro
    4. molybdopterin cofactor binding Source: InterPro
    5. sulfite oxidase activity Source: Reactome

    GO - Biological processi

    1. cellular nitrogen compound metabolic process Source: Reactome
    2. small molecule metabolic process Source: Reactome
    3. sulfide oxidation, using sulfide:quinone oxidoreductase Source: Reactome
    4. sulfur amino acid catabolic process Source: Reactome
    5. sulfur amino acid metabolic process Source: Reactome

    Keywords - Molecular functioni

    Oxidoreductase

    Keywords - Ligandi

    Heme, Iron, Metal-binding, Molybdenum

    Enzyme and pathway databases

    BioCyciMetaCyc:HS06627-MONOMER.
    ReactomeiREACT_116010. 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
    ProteomesiUP000005640: Chromosome 12

    Organism-specific databases

    HGNCiHGNC:11460. SUOX.

    Subcellular locationi

    GO - Cellular componenti

    1. mitochondrial intermembrane space Source: UniProtKB-SubCell
    2. mitochondrial matrix Source: Reactome

    Keywords - Cellular componenti

    Mitochondrion

    Pathology & Biotechi

    Involvement in diseasei

    Isolated sulfite oxidase deficiency (ISOD) [MIM:272300]: Characterized by neurological abnormalities including multicystic leukoencephalopathy with brain atrophy. Patients often suffer from seizures. Often leads to death at an early age.5 Publications
    Note: The disease is caused by mutations affecting the gene represented in this entry.
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti217 – 2171R → Q in ISOD; 2% of activity. 3 Publications
    VAR_002200
    Natural varianti258 – 2581I → L in ISOD. 1 Publication
    VAR_015724
    Natural varianti265 – 2651A → D in ISOD. 2 Publications
    VAR_002201
    Natural varianti268 – 2681R → Q in ISOD. 1 Publication
    VAR_015725
    Natural varianti362 – 3621G → S in ISOD. 1 Publication
    VAR_015726
    Natural varianti366 – 3661R → H in ISOD. 1 Publication
    VAR_015727
    Natural varianti379 – 3791K → R in ISOD. 1 Publication
    VAR_015728
    Natural varianti396 – 3961Q → R in ISOD. 1 Publication
    VAR_015729
    Natural varianti427 – 4271S → Y in ISOD. 2 Publications
    VAR_002202
    Natural varianti450 – 4501W → R in ISOD. 1 Publication
    VAR_015730
    Natural varianti530 – 5301G → D in ISOD. 1 Publication
    VAR_002203

    Keywords - Diseasei

    Disease mutation

    Organism-specific databases

    MIMi272300. phenotype.
    Orphaneti99731. Isolated sulfite oxidase deficiency.
    PharmGKBiPA36250.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 7979MitochondrionBy similarityAdd
    BLAST
    Chaini80 – 545466Sulfite oxidase, mitochondrialPRO_0000006481Add
    BLAST

    Proteomic databases

    MaxQBiP51687.
    PaxDbiP51687.
    PRIDEiP51687.

    PTM databases

    PhosphoSiteiP51687.

    Expressioni

    Gene expression databases

    ArrayExpressiP51687.
    BgeeiP51687.
    CleanExiHS_SUOX.
    GenevestigatoriP51687.

    Organism-specific databases

    HPAiHPA038209.

    Interactioni

    Subunit structurei

    Homodimer.By similarity

    Protein-protein interaction databases

    BioGridi112690. 3 interactions.
    IntActiP51687. 2 interactions.
    STRINGi9606.ENSP00000266971.

    Structurei

    Secondary structure

    1
    545
    Legend: HelixTurnBeta strand
    Show more details
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Turni96 – 983
    Beta strandi99 – 1046
    Beta strandi107 – 1104
    Turni112 – 1143
    Helixi115 – 1173
    Helixi122 – 1265
    Turni127 – 1304
    Beta strandi131 – 1333
    Helixi134 – 1374
    Helixi141 – 1433
    Helixi146 – 1538
    Beta strandi156 – 1594

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    EntryMethodResolution (Å)ChainPositionsPDBsum
    1MJ4X-ray1.20A79-160[»]
    ProteinModelPortaliP51687.
    SMRiP51687. Positions 81-543.
    ModBaseiSearch...
    MobiDBiSearch...

    Miscellaneous databases

    EvolutionaryTraceiP51687.

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Domaini82 – 16180Cytochrome b5 heme-bindingPROSITE-ProRule annotationAdd
    BLAST

    Region

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Regioni165 – 17410HingeBy similarity
    Regioni175 – 401227Moco domainBy similarityAdd
    BLAST
    Regioni215 – 2195Molybdopterin-bindingBy similarity
    Regioni377 – 3793Molybdopterin-bindingBy similarity
    Regioni402 – 538137HomodimerizationBy similarityAdd
    BLAST

    Sequence similaritiesi

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

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG2041.
    HOGENOMiHOG000252609.
    HOVERGENiHBG017865.
    InParanoidiP51687.
    KOiK00387.
    OMAiAVHNQSH.
    OrthoDBiEOG7P8P7Z.
    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.
    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

    MLLLHRAVVL RLQQACRLKS IPSRICIQAC STNDSFQPQR PSLTFSGDNS    50
    STQGWRVMGT LLGLGAVLAY QDHRCRAAQE STHIYTKEEV SSHTSPETGI 100
    WVTLGSEVFD VTEFVDLHPG GPSKLMLAAG GPLEPFWALY AVHNQSHVRE 150
    LLAQYKIGEL NPEDKVAPTV ETSDPYADDP VRHPALKVNS QRPFNAEPPP 200
    ELLTENYITP NPIFFTRNHL PVPNLDPDTY RLHVVGAPGG QSLSLSLDDL 250
    HNFPRYEITV TLQCAGNRRS EMTQVKEVKG LEWRTGAIST ARWAGARLCD 300
    VLAQAGHQLC ETEAHVCFEG LDSDPTGTAY GASIPLARAM DPEAEVLLAY 350
    EMNGQPLPRD HGFPVRVVVP GVVGARHVKW LGRVSVQPEE SYSHWQRRDY 400
    KGFSPSVDWE TVDFDSAPSI QELPVQSAIT EPRDGETVES GEVTIKGYAW 450
    SGGGRAVIRV DVSLDGGLTW QVAKLDGEEQ RPRKAWAWRL WQLKAPVPAG 500
    QKELNIVCKA VDDGYNVQPD TVAPIWNLRG VLSNAWHRVH VYVSP 545
    Length:545
    Mass (Da):60,283
    Last modified:July 10, 2007 - v2
    Checksum:i39B842C55D39E11F
    GO

    Sequence cautioni

    The sequence AAA74886.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.
    The sequence AAL08048.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti217 – 2171R → Q in ISOD; 2% of activity. 3 Publications
    VAR_002200
    Natural varianti258 – 2581I → L in ISOD. 1 Publication
    VAR_015724
    Natural varianti265 – 2651A → D in ISOD. 2 Publications
    VAR_002201
    Natural varianti268 – 2681R → Q in ISOD. 1 Publication
    VAR_015725
    Natural varianti362 – 3621G → S in ISOD. 1 Publication
    VAR_015726
    Natural varianti366 – 3661R → H in ISOD. 1 Publication
    VAR_015727
    Natural varianti379 – 3791K → R in ISOD. 1 Publication
    VAR_015728
    Natural varianti396 – 3961Q → R in ISOD. 1 Publication
    VAR_015729
    Natural varianti427 – 4271S → Y in ISOD. 2 Publications
    VAR_002202
    Natural varianti450 – 4501W → R in ISOD. 1 Publication
    VAR_015730
    Natural varianti530 – 5301G → D in ISOD. 1 Publication
    VAR_002203

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    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.
    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.3. human.

    Polymorphism databases

    DMDMi152031695.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    L31573 mRNA. Translation: AAA74886.1 . Different initiation.
    BC065193 mRNA. Translation: AAH65193.2 .
    AY056018 Genomic DNA. Translation: AAL08048.1 . Different initiation.
    CCDSi CCDS8901.2.
    PIRi S55874.
    RefSeqi NP_000447.2. NM_000456.2.
    NP_001027558.1. NM_001032386.1.
    NP_001027559.1. NM_001032387.1.
    UniGenei Hs.558403.

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    Entry Method Resolution (Å) Chain Positions PDBsum
    1MJ4 X-ray 1.20 A 79-160 [» ]
    ProteinModelPortali P51687.
    SMRi P51687. Positions 81-543.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 112690. 3 interactions.
    IntActi P51687. 2 interactions.
    STRINGi 9606.ENSP00000266971.

    PTM databases

    PhosphoSitei P51687.

    Polymorphism databases

    DMDMi 152031695.

    Proteomic databases

    MaxQBi P51687.
    PaxDbi P51687.
    PRIDEi P51687.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000266971 ; ENSP00000266971 ; ENSG00000139531 .
    ENST00000356124 ; ENSP00000348440 ; ENSG00000139531 .
    ENST00000394109 ; ENSP00000377668 ; ENSG00000139531 .
    ENST00000394115 ; ENSP00000377674 ; ENSG00000139531 .
    ENST00000548274 ; ENSP00000450245 ; ENSG00000139531 .
    ENST00000550065 ; ENSP00000450264 ; ENSG00000139531 .
    GeneIDi 6821.
    KEGGi hsa:6821.
    UCSCi uc001six.3. human.

    Organism-specific databases

    CTDi 6821.
    GeneCardsi GC12P056391.
    HGNCi HGNC:11460. SUOX.
    HPAi HPA038209.
    MIMi 272300. phenotype.
    606887. gene.
    neXtProti NX_P51687.
    Orphaneti 99731. Isolated sulfite oxidase deficiency.
    PharmGKBi PA36250.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG2041.
    HOGENOMi HOG000252609.
    HOVERGENi HBG017865.
    InParanoidi P51687.
    KOi K00387.
    OMAi AVHNQSH.
    OrthoDBi EOG7P8P7Z.
    PhylomeDBi P51687.
    TreeFami TF300905.

    Enzyme and pathway databases

    UniPathwayi UPA00096 .
    BioCyci MetaCyc:HS06627-MONOMER.
    Reactomei REACT_116010. Sulfide oxidation to sulfate.
    SABIO-RK P51687.

    Miscellaneous databases

    EvolutionaryTracei P51687.
    GeneWikii Sulfite_oxidase.
    GenomeRNAii 6821.
    NextBioi 26639.
    PROi P51687.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi P51687.
    Bgeei P51687.
    CleanExi HS_SUOX.
    Genevestigatori P51687.

    Family and domain databases

    Gene3Di 2.60.40.650. 1 hit.
    3.10.120.10. 1 hit.
    3.90.420.10. 1 hit.
    InterProi IPR001199. 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 ]
    Pfami PF00173. Cyt-b5. 1 hit.
    PF03404. Mo-co_dimer. 1 hit.
    PF00174. Oxidored_molyb. 1 hit.
    [Graphical view ]
    PRINTSi PR00363. CYTOCHROMEB5.
    PR00407. EUMOPTERIN.
    SUPFAMi SSF55856. SSF55856. 1 hit.
    SSF56524. SSF56524. 1 hit.
    SSF81296. SSF81296. 1 hit.
    PROSITEi PS00191. CYTOCHROME_B5_1. 1 hit.
    PS50255. CYTOCHROME_B5_2. 1 hit.
    PS00559. MOLYBDOPTERIN_EUK. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. Cited for: NUCLEOTIDE SEQUENCE [MRNA].
      Tissue: Liver.
    2. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Pancreas.
    3. "Genomic DNA sequence of human sulfite oxidase SUOX."
      Coyne K.E., Johnson J.L., Rajagopalan K.V.
      Submitted (SEP-2001) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 18-545.
    4. "The 1.2 A structure of the human sulfite oxidase cytochrome b(5) domain."
      Rudolph M.J., Johnson J.L., Rajagopalan K.V., Kisker C.
      Acta Crystallogr. D 59:1183-1191(2003) [PubMed] [Europe PMC] [Abstract]
      Cited for: X-RAY CRYSTALLOGRAPHY (1.2 ANGSTROMS) OF 79-160 IN COMPLEX WITH HEME, COFACTOR.
    5. "Molecular basis of sulfite oxidase deficiency from the structure of sulfite oxidase."
      Kisker C., Schindelin H., Pacheco A., Wehbi W.A., Garrett R.M., Rajagopalan K.V., Enemark J.H., Rees D.C.
      Cell 91:973-983(1997) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANTS ISOD GLN-217; ASP-265; TYR-427 AND ASP-530.
    6. "Human sulfite oxidase R160Q: identification of the mutation in a sulfite oxidase-deficient patient and expression and characterization of the mutant enzyme."
      Garrett R.M., Johnson J.L., Graf T.N., Feigenbaum A., Rajagopalan K.V.
      Proc. Natl. Acad. Sci. U.S.A. 95:6394-6398(1998) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT ISOD GLN-217.
    7. "Isolated sulfite oxidase deficiency: review of two cases in one family."
      Edwards M.C., Johnson J.L., Marriage B., Graf T.N., Coyne K.E., Rajagopalan K.V., MacDonald I.M.
      Ophthalmology 106:1957-1961(1999) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANTS ISOD ASP-265 AND TYR-427.
    8. "Isolated sulfite oxidase deficiency: identification of 12 novel SUOX mutations in 10 patients."
      Johnson J.L., Coyne K.E., Garrett R.M., Zabot M.-T., Dorche C., Kisker C., Rajagopalan K.V.
      Hum. Mutat. 20:74-74(2002) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANTS ISOD LEU-258; GLN-268; SER-362; HIS-366; ARG-379; ARG-396 AND ARG-450.
    9. "A novel mutation in neonatal isolated sulphite oxidase deficiency."
      Lee H.F., Mak B.S., Chi C.S., Tsai C.R., Chen C.H., Shu S.G.
      Neuropediatrics 33:174-179(2002) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT ISOD GLN-217.

    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: October 1, 2014
    This is version 157 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

    External Data

    Dasty 3