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O09173 (HGD_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 111. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Homogentisate 1,2-dioxygenase

EC=1.13.11.5
Alternative name(s):
Homogentisate oxygenase
Homogentisic acid oxidase
Homogentisicase
Gene names
Name:Hgd
Synonyms:Aku, Hgo
OrganismMus musculus (Mouse) [Reference proteome]
Taxonomic identifier10090 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus

Protein attributes

Sequence length445 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Catalytic activity

Homogentisate + O2 = 4-maleylacetoacetate.

Cofactor

Iron.

Pathway

Amino-acid degradation; L-phenylalanine degradation; acetoacetate and fumarate from L-phenylalanine: step 4/6.

Subunit structure

Homohexamer arranged as a dimer of trimers By similarity.

Involvement in disease

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.

Sequence similarities

Belongs to the homogentisate dioxygenase family.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 445445Homogentisate 1,2-dioxygenase
PRO_0000220241

Sites

Metal binding3351Iron By similarity
Metal binding3411Iron By similarity
Metal binding3711Iron By similarity

Amino acid modifications

Modified residue981N6-acetyllysine By similarity
Modified residue4141N6-succinyllysine Ref.5

Experimental info

Sequence conflict2671A → T in AAC53224. Ref.1

Sequences

Sequence LengthMass (Da)Tools
O09173 [UniParc].

Last modified July 27, 2011. Version 2.
Checksum: 27DEB34D38DB1C25

FASTA44549,960
        10         20         30         40         50         60 
MAELKYISGF GNECASEDPR CPGSLPKGQN NPQVCPYNLY AEQLSGSAFT CPRNTNKRSW 

        70         80         90        100        110        120 
LYRILPSVSH KPFESIDQGH VTHNWDEVGP DPNQLRWKPF EIPKASEKKV DFVSGLYTLC 

       130        140        150        160        170        180 
GAGDIKSNNG LAVHIFLCNS SMENRCFYNS DGDFLIVPQK GKLLIYTEFG KMSLQPNEIC 

       190        200        210        220        230        240 
VIQRGMRFSV DVFEETRGYI LEVYGVHFEL PDLGPIGANG LANPRDFLIP VAWYEDRRVP 

       250        260        270        280        290        300 
GGYTVINKFQ GKLFACKQDV SPFNVVAWHG NYTPYKYNLE NFMVINAVAF DHADPSIFTV 

       310        320        330        340        350        360 
LTAKSLRPGV AIADFVIFPP RWGVADKTFR PPYYHRNCMS EFMGLIKGHY EAKQGGFLPG 

       370        380        390        400        410        420 
GGSLHSAMTP HGPDADCFEK ASKAKLEPER IADGTMAFMF ESSLSLAVTK WGLKTCSCLD 

       430        440 
ENYYKCWEPL RSHFTPNSRS PTEPK 

« Hide

References

« Hide 'large scale' references
[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.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
U58988 mRNA. Translation: AAC53224.1.
CH466521 Genomic DNA. Translation: EDK97961.1.
BC055029 mRNA. Translation: AAH55029.1.
CCDSCCDS49849.1.
RefSeqNP_038575.2. NM_013547.3.
UniGeneMm.157442.

3D structure databases

ProteinModelPortalO09173.
SMRO09173. Positions 2-440.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

IntActO09173. 5 interactions.
MINTMINT-1857911.
STRING10090.ENSMUSP00000023519.

PTM databases

PhosphoSiteO09173.

Proteomic databases

MaxQBO09173.
PaxDbO09173.
PRIDEO09173.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENSMUST00000160847; ENSMUSP00000125492; ENSMUSG00000022821.
GeneID15233.
KEGGmmu:15233.
UCSCuc007zeg.2. mouse.

Organism-specific databases

CTD3081.
MGIMGI:96078. Hgd.

Phylogenomic databases

eggNOGCOG3508.
GeneTreeENSGT00390000004601.
HOGENOMHOG000139824.
HOVERGENHBG005965.
InParanoidQ7TPP2.
KOK00451.
OMARCFYNSD.
OrthoDBEOG7PP58Z.
PhylomeDBO09173.
TreeFamTF300490.

Enzyme and pathway databases

SABIO-RKO09173.
UniPathwayUPA00139; UER00339.

Gene expression databases

BgeeO09173.
CleanExMM_HGD.
GenevestigatorO09173.

Family and domain databases

Gene3D2.60.120.10. 2 hits.
InterProIPR005708. Homogentis_dOase.
IPR014710. RmlC-like_jellyroll.
IPR011051. RmlC_Cupin.
[Graphical view]
PANTHERPTHR11056. PTHR11056. 1 hit.
PfamPF04209. HgmA. 1 hit.
[Graphical view]
SUPFAMSSF51182. SSF51182. 1 hit.
TIGRFAMsTIGR01015. hmgA. 1 hit.
ProtoNetSearch...

Other

NextBio287827.
PROO09173.
SOURCESearch...

Entry information

Entry nameHGD_MOUSE
AccessionPrimary (citable) accession number: O09173
Secondary accession number(s): Q7TPP2
Entry history
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 statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families

PATHWAY comments

Index of metabolic and biosynthesis pathways

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot