ID ECHD1_PONAB Reviewed; 301 AA.
AC Q5R4W0;
DT 23-JAN-2007, integrated into UniProtKB/Swiss-Prot.
DT 21-DEC-2004, sequence version 1.
DT 24-JAN-2024, entry version 81.
DE RecName: Full=Ethylmalonyl-CoA decarboxylase;
DE EC=4.1.1.94 {ECO:0000250|UniProtKB:Q9D9V3};
DE AltName: Full=Enoyl-CoA hydratase domain-containing protein 1;
DE AltName: Full=Methylmalonyl-CoA decarboxylase;
DE Short=MMCD;
GN Name=ECHDC1;
OS Pongo abelii (Sumatran orangutan) (Pongo pygmaeus abelii).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae;
OC Pongo.
OX NCBI_TaxID=9601;
RN [1]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC TISSUE=Brain cortex;
RG The German cDNA consortium;
RL Submitted (NOV-2004) to the EMBL/GenBank/DDBJ databases.
CC -!- FUNCTION: Decarboxylates ethylmalonyl-CoA, a potentially toxic
CC metabolite, to form butyryl-CoA, suggesting it might be involved in
CC metabolite proofreading. Acts preferentially on (S)-ethylmalonyl-CoA
CC but has also some activity on the (R)-isomer. Also has methylmalonyl-
CC CoA decarboxylase activity at lower level.
CC {ECO:0000250|UniProtKB:Q9D9V3}.
CC -!- CATALYTIC ACTIVITY:
CC Reaction=(2S)-ethylmalonyl-CoA + H(+) = butanoyl-CoA + CO2;
CC Xref=Rhea:RHEA:32131, ChEBI:CHEBI:15378, ChEBI:CHEBI:16526,
CC ChEBI:CHEBI:57371, ChEBI:CHEBI:60909; EC=4.1.1.94;
CC Evidence={ECO:0000250|UniProtKB:Q9D9V3};
CC PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:32132;
CC Evidence={ECO:0000250|UniProtKB:Q9D9V3};
CC -!- CATALYTIC ACTIVITY:
CC Reaction=(S)-methylmalonyl-CoA + H(+) = CO2 + propanoyl-CoA;
CC Xref=Rhea:RHEA:61340, ChEBI:CHEBI:15378, ChEBI:CHEBI:16526,
CC ChEBI:CHEBI:57327, ChEBI:CHEBI:57392; EC=4.1.1.94;
CC Evidence={ECO:0000250|UniProtKB:Q9D9V3};
CC PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:61341;
CC Evidence={ECO:0000250|UniProtKB:Q9D9V3};
CC -!- CATALYTIC ACTIVITY:
CC Reaction=(2R)-ethylmalonyl-CoA + H(+) = butanoyl-CoA + CO2;
CC Xref=Rhea:RHEA:59540, ChEBI:CHEBI:15378, ChEBI:CHEBI:16526,
CC ChEBI:CHEBI:57371, ChEBI:CHEBI:85316; EC=4.1.1.94;
CC Evidence={ECO:0000250|UniProtKB:Q9D9V3};
CC PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:59541;
CC Evidence={ECO:0000250|UniProtKB:Q9D9V3};
CC -!- SUBCELLULAR LOCATION: Cytoplasm, cytosol
CC {ECO:0000250|UniProtKB:Q9D9V3}.
CC -!- SIMILARITY: Belongs to the enoyl-CoA hydratase/isomerase family.
CC {ECO:0000305}.
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DR EMBL; CR861131; CAH93206.1; -; mRNA.
DR EMBL; CR925967; CAI29618.1; -; mRNA.
DR RefSeq; NP_001126886.1; NM_001133414.1.
DR RefSeq; XP_009240496.1; XM_009242221.1.
DR AlphaFoldDB; Q5R4W0; -.
DR SMR; Q5R4W0; -.
DR STRING; 9601.ENSPPYP00000024266; -.
DR Ensembl; ENSPPYT00000019781.2; ENSPPYP00000019030.2; ENSPPYG00000016995.3.
DR GeneID; 100173900; -.
DR KEGG; pon:100173900; -.
DR CTD; 55862; -.
DR eggNOG; KOG1680; Eukaryota.
DR GeneTree; ENSGT00880000138038; -.
DR InParanoid; Q5R4W0; -.
DR OrthoDB; 50856at2759; -.
DR Proteomes; UP000001595; Chromosome 6.
DR GO; GO:0005829; C:cytosol; ISS:UniProtKB.
DR GO; GO:0016831; F:carboxy-lyase activity; ISS:UniProtKB.
DR GO; GO:0004492; F:methyl/ethyl malonyl-CoA decarboxylase activity; IEA:UniProtKB-EC.
DR CDD; cd06558; crotonase-like; 1.
DR InterPro; IPR029045; ClpP/crotonase-like_dom_sf.
DR InterPro; IPR018376; Enoyl-CoA_hyd/isom_CS.
DR InterPro; IPR001753; Enoyl-CoA_hydra/iso.
DR PANTHER; PTHR11941; ENOYL-COA HYDRATASE-RELATED; 1.
DR PANTHER; PTHR11941:SF27; ETHYLMALONYL-COA DECARBOXYLASE; 1.
DR Pfam; PF00378; ECH_1; 1.
DR SUPFAM; SSF52096; ClpP/crotonase; 1.
DR PROSITE; PS00166; ENOYL_COA_HYDRATASE; 1.
PE 2: Evidence at transcript level;
KW Acetylation; Cytoplasm; Lyase; Reference proteome.
FT CHAIN 1..301
FT /note="Ethylmalonyl-CoA decarboxylase"
FT /id="PRO_0000273248"
FT MOD_RES 211
FT /note="N6-acetyllysine; alternate"
FT /evidence="ECO:0000250|UniProtKB:Q9D9V3"
FT MOD_RES 211
FT /note="N6-succinyllysine; alternate"
FT /evidence="ECO:0000250|UniProtKB:Q9D9V3"
FT MOD_RES 295
FT /note="N6-succinyllysine"
FT /evidence="ECO:0000250|UniProtKB:Q9D9V3"
SQ SEQUENCE 301 AA; 32985 MW; EC74C79DA2FB6FD8 CRC64;
MAKSLLKTSS LSGRTKLLHQ TGLSLYSTSH GFYEEEVKKT LQQFPGGSID LQKEDNGIGI
LTLNNPSKMN AFSGVMMLQL LEKVIELENW TEGKGLIVRG AKNTFSSGSD LNAVKSLGTP
EDGMAVCMFM QNTLTRFMRL PLISVALVQG WALGGGAEFT TACDFRLMTP ESKIRFVHKE
MGIIPSWGGT TRLVEIIGSR QALKVLSGAL KLDSKNALNI GMVEEVLQSS DETKSLEEAQ
EWLKQFIQGP PEVIRALKKS VCSGRELYLE EALQNERDLL GTVWGGPANL EAIAKKGKFN
K
//
