HGD_CAEEL
ID HGD_CAEEL Reviewed; 437 AA.
AC Q9Y041; O62087; Q9NJP3;
DT 30-MAY-2000, integrated into UniProtKB/Swiss-Prot.
DT 27-APR-2001, sequence version 2.
DT 03-AUG-2022, entry version 153.
DE RecName: Full=Homogentisate 1,2-dioxygenase;
DE EC=1.13.11.5;
DE AltName: Full=Homogentisate oxygenase;
DE AltName: Full=Homogentisic acid oxidase;
DE AltName: Full=Homogentisicase;
GN Name=hgo-1 {ECO:0000312|WormBase:W06D4.1};
GN ORFNames=W06D4.1 {ECO:0000312|WormBase:W06D4.1};
OS Caenorhabditis elegans.
OC Eukaryota; Metazoa; Ecdysozoa; Nematoda; Chromadorea; Rhabditida;
OC Rhabditina; Rhabditomorpha; Rhabditoidea; Rhabditidae; Peloderinae;
OC Caenorhabditis.
OX NCBI_TaxID=6239;
RN [1]
RP NUCLEOTIDE SEQUENCE [GENOMIC DNA].
RC STRAIN=Bristol N2;
RA Schmidt S.R., Werner E., Mueller C.R., Kress W.;
RT "Cloning and characterization of the homogentisate 1,2-dioxygenase gene in
RT A. thaliana and C. elegans.";
RL Submitted (DEC-1996) to the EMBL/GenBank/DDBJ databases.
RN [2]
RP NUCLEOTIDE SEQUENCE [MRNA].
RC STRAIN=Bristol N2; TISSUE=Embryo;
RA Schmidt S.R., Werner E., Mueller C.R., Kress W.;
RT "Sequence homology of HGO genes in eukaryotic organisms.";
RL Submitted (MAR-1999) to the EMBL/GenBank/DDBJ databases.
RN [3]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RC STRAIN=Bristol N2;
RX PubMed=9851916; DOI=10.1126/science.282.5396.2012;
RG The C. elegans sequencing consortium;
RT "Genome sequence of the nematode C. elegans: a platform for investigating
RT biology.";
RL Science 282:2012-2018(1998).
RN [4]
RP FUNCTION, TISSUE SPECIFICITY, DISRUPTION PHENOTYPE, AND MUTAGENESIS OF
RP GLY-173.
RX PubMed=18227072; DOI=10.1074/jbc.m708341200;
RA Fisher A.L., Page K.E., Lithgow G.J., Nash L.;
RT "The Caenorhabditis elegans K10C2.4 gene encodes a member of the
RT fumarylacetoacetate hydrolase family: a Caenorhabditis elegans model of
RT type I tyrosinemia.";
RL J. Biol. Chem. 283:9127-9135(2008).
CC -!- FUNCTION: Plays a role in the tyrosine degradation pathway.
CC {ECO:0000305|PubMed:18227072}.
CC -!- CATALYTIC ACTIVITY:
CC Reaction=homogentisate + O2 = 4-maleylacetoacetate + H(+);
CC Xref=Rhea:RHEA:15449, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379,
CC ChEBI:CHEBI:16169, ChEBI:CHEBI:17105; EC=1.13.11.5;
CC -!- COFACTOR:
CC Name=Fe cation; Xref=ChEBI:CHEBI:24875;
CC -!- PATHWAY: Amino-acid degradation; L-phenylalanine degradation;
CC acetoacetate and fumarate from L-phenylalanine: step 4/6.
CC -!- TISSUE SPECIFICITY: Expressed in the hypodermis and intestine.
CC {ECO:0000269|PubMed:18227072}.
CC -!- DISRUPTION PHENOTYPE: RNAi-mediated knockdown together with fah-1 RNAi
CC rescues the impaired growth and fertility defects in the single fah-1
CC RNAi mutant. {ECO:0000269|PubMed:18227072}.
CC -!- SIMILARITY: Belongs to the homogentisate dioxygenase family.
CC {ECO:0000305}.
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DR EMBL; AF136150; AAF61419.1; -; Genomic_DNA.
DR EMBL; U95181; AAD00776.1; -; mRNA.
DR EMBL; BX284601; CAA22255.4; -; Genomic_DNA.
DR EMBL; Z93778; CAA22255.4; JOINED; Genomic_DNA.
DR PIR; T19626; T19626.
DR PIR; T37469; T37469.
DR RefSeq; NP_492433.1; NM_060032.6.
DR AlphaFoldDB; Q9Y041; -.
DR SMR; Q9Y041; -.
DR BioGRID; 38158; 5.
DR STRING; 6239.W06D4.1; -.
DR iPTMnet; Q9Y041; -.
DR EPD; Q9Y041; -.
DR PaxDb; Q9Y041; -.
DR PeptideAtlas; Q9Y041; -.
DR EnsemblMetazoa; W06D4.1.1; W06D4.1.1; WBGene00001843.
DR GeneID; 172726; -.
DR KEGG; cel:CELE_W06D4.1; -.
DR UCSC; W06D4.1; c. elegans.
DR CTD; 172726; -.
DR WormBase; W06D4.1; CE29602; WBGene00001843; hgo-1.
DR eggNOG; KOG1417; Eukaryota.
DR GeneTree; ENSGT00390000004601; -.
DR HOGENOM; CLU_027174_0_0_1; -.
DR InParanoid; Q9Y041; -.
DR OMA; FMFETRW; -.
DR OrthoDB; 795654at2759; -.
DR PhylomeDB; Q9Y041; -.
DR Reactome; R-CEL-8963684; Tyrosine catabolism.
DR UniPathway; UPA00139; UER00339.
DR PRO; PR:Q9Y041; -.
DR Proteomes; UP000001940; Chromosome I.
DR Bgee; WBGene00001843; Expressed in material anatomical entity and 5 other tissues.
DR GO; GO:0005737; C:cytoplasm; HDA:WormBase.
DR GO; GO:0055120; C:striated muscle dense body; HDA:WormBase.
DR GO; GO:0004411; F:homogentisate 1,2-dioxygenase activity; IBA:GO_Central.
DR GO; GO:0046872; F:metal ion binding; IEA:UniProtKB-KW.
DR GO; GO:0006559; P:L-phenylalanine catabolic process; IBA:GO_Central.
DR GO; GO:0006572; P:tyrosine catabolic process; IEA:UniProtKB-KW.
DR Gene3D; 2.60.120.10; -; 1.
DR InterPro; IPR005708; Homogentis_dOase.
DR InterPro; IPR014710; RmlC-like_jellyroll.
DR InterPro; IPR011051; RmlC_Cupin_sf.
DR PANTHER; PTHR11056; PTHR11056; 1.
DR Pfam; PF04209; HgmA; 1.
DR SUPFAM; SSF51182; SSF51182; 1.
DR TIGRFAMs; TIGR01015; hmgA; 1.
PE 1: Evidence at protein level;
KW Dioxygenase; Iron; Metal-binding; Oxidoreductase; Phenylalanine catabolism;
KW Reference proteome; Tyrosine catabolism.
FT CHAIN 1..437
FT /note="Homogentisate 1,2-dioxygenase"
FT /id="PRO_0000220243"
FT REGION 15..34
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT BINDING 336
FT /ligand="Fe cation"
FT /ligand_id="ChEBI:CHEBI:24875"
FT /evidence="ECO:0000250"
FT BINDING 342
FT /ligand="Fe cation"
FT /ligand_id="ChEBI:CHEBI:24875"
FT /evidence="ECO:0000250"
FT BINDING 372
FT /ligand="Fe cation"
FT /ligand_id="ChEBI:CHEBI:24875"
FT /evidence="ECO:0000250"
FT MUTAGEN 173
FT /note="G->R: Suppresses fah-1 RNAi-mediated toxicity."
FT /evidence="ECO:0000269|PubMed:18227072"
FT CONFLICT 156
FT /note="D -> N (in Ref. 2; AAD00776)"
FT /evidence="ECO:0000305"
FT CONFLICT 176
FT /note="L -> P (in Ref. 2; AAD00776)"
FT /evidence="ECO:0000305"
FT CONFLICT 193
FT /note="V -> G (in Ref. 2; AAD00776)"
FT /evidence="ECO:0000305"
FT CONFLICT 242
FT /note="V -> G (in Ref. 2; AAD00776)"
FT /evidence="ECO:0000305"
FT CONFLICT 265
FT /note="D -> E (in Ref. 2; AAD00776)"
FT /evidence="ECO:0000305"
FT CONFLICT 433
FT /note="F -> Y (in Ref. 2; AAD00776)"
FT /evidence="ECO:0000305"
SQ SEQUENCE 437 AA; 49239 MW; C14E7077C7CF9703 CRC64;
MSEFDELKYL TGFGNEHATS DPRVPDALPV GQNSPQKCSH GLYAEQLSGT AFTAPRSQNQ
RSWLYRIRPS VIHRPFEAMK ENDQHWTNNF SSIPPNPNQY RWNPFPLPTK EGVTFVDNLY
TVCGGGDVIS RTGLAIHQFS CNASMEHTAM YNSDGDFLIV PQQGALEITT EFGRLLVNPQ
EIAVIPQGIR FSVAVRGPSR GYILEVYGTH FQLPDLGPIG ANGLANPRDF EAPVAWFEDL
DVEFTIINKY QGSWFQAKQG HSPFDVVGWH GNYVPYKYDL KKFMVINTVS FDHCDPSIFT
VLTAPSVKHG TAIADFVIFP PRWGCADNTF RPPYYHRNCM SEYMGLITGC YEAKEGGFKP
GGGSLHSMMT PHGPDFNCFE MASNADLKPQ RVAEGTMSFM FESSLNMAIT NWAVYQNVDK
DYYKDWQPLK KHFTMPK
