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O09173

- HGD_MOUSE

UniProt

O09173 - HGD_MOUSE

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Protein

Homogentisate 1,2-dioxygenase

Gene
Hgd, Aku, Hgo
Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 4 out of 5 - Experimental evidence at protein leveli

Functioni

Catalytic activityi

Homogentisate + O2 = 4-maleylacetoacetate.

Cofactori

Iron.

Pathwayi

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi335 – 3351Iron By similarity
Metal bindingi341 – 3411Iron By similarity
Metal bindingi371 – 3711Iron By 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
Complete GO annotation...

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-acetyllysine By 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.

Family & Domainsi

Sequence similaritiesi

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

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti267 – 2671A → T in AAC53224. 1 Publication

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

« Hide 'large scale' publications
  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: July 9, 2014
This is version 111 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

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