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O09173

- HGD_MOUSE

UniProt

O09173 - HGD_MOUSE

Protein

Homogentisate 1,2-dioxygenase

Gene

Hgd

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 4 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 112 (01 Oct 2014)
      Sequence version 2 (27 Jul 2011)
      Previous versions | rss
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    Functioni

    Catalytic activityi

    Homogentisate + O2 = 4-maleylacetoacetate.

    Cofactori

    Iron.

    Pathwayi

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Metal bindingi335 – 3351IronBy similarity
    Metal bindingi341 – 3411IronBy similarity
    Metal bindingi371 – 3711IronBy similarity

    GO - Molecular functioni

    1. homogentisate 1,2-dioxygenase activity Source: UniProtKB-EC
    2. metal ion binding Source: UniProtKB-KW

    GO - Biological processi

    1. cellular amino acid metabolic process Source: MGI
    2. L-phenylalanine catabolic process Source: UniProtKB-UniPathway
    3. tyrosine catabolic process Source: UniProtKB-KW

    Keywords - Molecular functioni

    Dioxygenase, Oxidoreductase

    Keywords - Biological processi

    Phenylalanine catabolism, Tyrosine catabolism

    Keywords - Ligandi

    Iron, Metal-binding

    Enzyme and pathway databases

    SABIO-RKO09173.
    UniPathwayiUPA00139; UER00339.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Homogentisate 1,2-dioxygenase (EC:1.13.11.5)
    Alternative name(s):
    Homogentisate oxygenase
    Homogentisic acid oxidase
    Homogentisicase
    Gene namesi
    Name:Hgd
    Synonyms:Aku, Hgo
    OrganismiMus musculus (Mouse)
    Taxonomic identifieri10090 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
    ProteomesiUP000000589: Chromosome 16

    Organism-specific databases

    MGIiMGI:96078. Hgd.

    Pathology & Biotechi

    Involvement in diseasei

    Defects in Hgd are the cause of alkaptonuria (aku). Aku is an autosomal recessive error of metabolism which is characterized by an increase in the level of homogentisic acid.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 445445Homogentisate 1,2-dioxygenasePRO_0000220241Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei98 – 981N6-acetyllysineBy similarity
    Modified residuei414 – 4141N6-succinyllysine1 Publication

    Keywords - PTMi

    Acetylation

    Proteomic databases

    MaxQBiO09173.
    PaxDbiO09173.
    PRIDEiO09173.

    PTM databases

    PhosphoSiteiO09173.

    Expressioni

    Gene expression databases

    BgeeiO09173.
    CleanExiMM_HGD.
    GenevestigatoriO09173.

    Interactioni

    Subunit structurei

    Homohexamer arranged as a dimer of trimers.By similarity

    Protein-protein interaction databases

    IntActiO09173. 5 interactions.
    MINTiMINT-1857911.
    STRINGi10090.ENSMUSP00000023519.

    Structurei

    3D structure databases

    ProteinModelPortaliO09173.
    SMRiO09173. Positions 2-440.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the homogentisate dioxygenase family.Curated

    Phylogenomic databases

    eggNOGiCOG3508.
    GeneTreeiENSGT00390000004601.
    HOGENOMiHOG000139824.
    HOVERGENiHBG005965.
    InParanoidiQ7TPP2.
    KOiK00451.
    OMAiRCFYNSD.
    OrthoDBiEOG7PP58Z.
    PhylomeDBiO09173.
    TreeFamiTF300490.

    Family and domain databases

    Gene3Di2.60.120.10. 2 hits.
    InterProiIPR005708. Homogentis_dOase.
    IPR014710. RmlC-like_jellyroll.
    IPR011051. RmlC_Cupin.
    [Graphical view]
    PANTHERiPTHR11056. PTHR11056. 1 hit.
    PfamiPF04209. HgmA. 1 hit.
    [Graphical view]
    SUPFAMiSSF51182. SSF51182. 1 hit.
    TIGRFAMsiTIGR01015. hmgA. 1 hit.

    Sequencei

    Sequence statusi: Complete.

    O09173-1 [UniParc]FASTAAdd to Basket

    « Hide

    MAELKYISGF GNECASEDPR CPGSLPKGQN NPQVCPYNLY AEQLSGSAFT    50
    CPRNTNKRSW LYRILPSVSH KPFESIDQGH VTHNWDEVGP DPNQLRWKPF 100
    EIPKASEKKV DFVSGLYTLC GAGDIKSNNG LAVHIFLCNS SMENRCFYNS 150
    DGDFLIVPQK GKLLIYTEFG KMSLQPNEIC VIQRGMRFSV DVFEETRGYI 200
    LEVYGVHFEL PDLGPIGANG LANPRDFLIP VAWYEDRRVP GGYTVINKFQ 250
    GKLFACKQDV SPFNVVAWHG NYTPYKYNLE NFMVINAVAF DHADPSIFTV 300
    LTAKSLRPGV AIADFVIFPP RWGVADKTFR PPYYHRNCMS EFMGLIKGHY 350
    EAKQGGFLPG GGSLHSAMTP HGPDADCFEK ASKAKLEPER IADGTMAFMF 400
    ESSLSLAVTK WGLKTCSCLD ENYYKCWEPL RSHFTPNSRS PTEPK 445
    Length:445
    Mass (Da):49,960
    Last modified:July 27, 2011 - v2
    Checksum:i27DEB34D38DB1C25
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti267 – 2671A → T in AAC53224. (PubMed:9069115)Curated

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    U58988 mRNA. Translation: AAC53224.1.
    CH466521 Genomic DNA. Translation: EDK97961.1.
    BC055029 mRNA. Translation: AAH55029.1.
    CCDSiCCDS49849.1.
    RefSeqiNP_038575.2. NM_013547.3.
    UniGeneiMm.157442.

    Genome annotation databases

    EnsembliENSMUST00000160847; ENSMUSP00000125492; ENSMUSG00000022821.
    GeneIDi15233.
    KEGGimmu:15233.
    UCSCiuc007zeg.2. mouse.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    U58988 mRNA. Translation: AAC53224.1 .
    CH466521 Genomic DNA. Translation: EDK97961.1 .
    BC055029 mRNA. Translation: AAH55029.1 .
    CCDSi CCDS49849.1.
    RefSeqi NP_038575.2. NM_013547.3.
    UniGenei Mm.157442.

    3D structure databases

    ProteinModelPortali O09173.
    SMRi O09173. Positions 2-440.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    IntActi O09173. 5 interactions.
    MINTi MINT-1857911.
    STRINGi 10090.ENSMUSP00000023519.

    PTM databases

    PhosphoSitei O09173.

    Proteomic databases

    MaxQBi O09173.
    PaxDbi O09173.
    PRIDEi O09173.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSMUST00000160847 ; ENSMUSP00000125492 ; ENSMUSG00000022821 .
    GeneIDi 15233.
    KEGGi mmu:15233.
    UCSCi uc007zeg.2. mouse.

    Organism-specific databases

    CTDi 3081.
    MGIi MGI:96078. Hgd.

    Phylogenomic databases

    eggNOGi COG3508.
    GeneTreei ENSGT00390000004601.
    HOGENOMi HOG000139824.
    HOVERGENi HBG005965.
    InParanoidi Q7TPP2.
    KOi K00451.
    OMAi RCFYNSD.
    OrthoDBi EOG7PP58Z.
    PhylomeDBi O09173.
    TreeFami TF300490.

    Enzyme and pathway databases

    UniPathwayi UPA00139 ; UER00339 .
    SABIO-RK O09173.

    Miscellaneous databases

    NextBioi 287827.
    PROi O09173.
    SOURCEi Search...

    Gene expression databases

    Bgeei O09173.
    CleanExi MM_HGD.
    Genevestigatori O09173.

    Family and domain databases

    Gene3Di 2.60.120.10. 2 hits.
    InterProi IPR005708. Homogentis_dOase.
    IPR014710. RmlC-like_jellyroll.
    IPR011051. RmlC_Cupin.
    [Graphical view ]
    PANTHERi PTHR11056. PTHR11056. 1 hit.
    Pfami PF04209. HgmA. 1 hit.
    [Graphical view ]
    SUPFAMi SSF51182. SSF51182. 1 hit.
    TIGRFAMsi TIGR01015. hmgA. 1 hit.
    ProtoNeti Search...

    Publicationsi

    1. "Cloning of the homogentisate 1,2-dioxygenase gene, the key enzyme of alkaptonuria in mouse."
      Schmidt S.R., Gehrig A., Koehler M.R., Schmid M., Mueller C.R., Kress W.
      Mamm. Genome 8:168-171(1997) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA], PARTIAL PROTEIN SEQUENCE.
      Strain: C57BL/6 X CBA.
      Tissue: Liver.
    2. Mural R.J., Adams M.D., Myers E.W., Smith H.O., Venter J.C.
      Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    3. "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].
      Strain: FVB/N.
      Tissue: Liver.
    4. "Murine liver homogentisate 1,2-dioxygenase. Purification to homogeneity and novel biochemical properties."
      Schmidt S.R., Muller C.R., Kress W.
      Eur. J. Biochem. 228:425-430(1995) [PubMed] [Europe PMC] [Abstract]
      Cited for: CHARACTERIZATION.
      Tissue: Liver.
    5. "SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways."
      Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.
      Mol. Cell 50:919-930(2013) [PubMed] [Europe PMC] [Abstract]
      Cited for: SUCCINYLATION [LARGE SCALE ANALYSIS] AT LYS-414, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
      Tissue: Liver.

    Entry informationi

    Entry nameiHGD_MOUSE
    AccessioniPrimary (citable) accession number: O09173
    Secondary accession number(s): Q7TPP2
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: May 30, 2000
    Last sequence update: July 27, 2011
    Last modified: October 1, 2014
    This is version 112 of the entry and version 2 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Direct protein sequencing, Reference proteome

    Documents

    1. MGD cross-references
      Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
    2. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    3. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3