Ethylmalonyl-CoA decarboxylase

Ethylmalonyl-CoA decarboxylase (EC 4.1.1.94) is an enzyme with systematic name (S)-ethylmalonyl-CoA carboxy-lyase (butanoyl-CoA-forming).[5] This enzyme catalyses the following chemical reaction

(S)-ethylmalonyl-CoA butanoyl-CoA + CO2
ECHDC1
Identifiers
AliasesECHDC1, HEL-S-76, MMCD, dJ351K20.2, ethylmalonyl-CoA decarboxylase 1
External IDsOMIM: 612136 MGI: 1277169 HomoloGene: 23106 GeneCards: ECHDC1
EC number7.2.4.3
Gene location (Human)
Chr.Chromosome 6 (human)[1]
Band6q22.33Start127,288,712 bp[1]
End127,343,609 bp[1]
Orthologs
SpeciesHumanMouse
Entrez

55862

52665

Ensembl

ENSG00000093144

ENSMUSG00000019883

UniProt

Q9NTX5

Q9D9V3

RefSeq (mRNA)

NM_001002030
NM_001105544
NM_001105545
NM_001139510
NM_018479

NM_001110195
NM_025855

RefSeq (protein)

NP_001002030
NP_001099014
NP_001099015
NP_001132982
NP_060949

NP_001103665
NP_080131

Location (UCSC)Chr 6: 127.29 – 127.34 MbChr 10: 29.31 – 29.35 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse
Ethylmalonyl-CoA decarboxylase
Identifiers
EC number4.1.1.94
Databases
IntEnzIntEnz view
BRENDABRENDA entry
ExPASyNiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
PRIAMprofile
PDB structuresRCSB PDB PDBe PDBsum

The vertebrate enzyme decarboxylates ethylmalonyl-CoA.

References

  1. GRCh38: Ensembl release 89: ENSG00000093144 - Ensembl, May 2017
  2. GRCm38: Ensembl release 89: ENSMUSG00000019883 - Ensembl, May 2017
  3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. Linster CL, Noël G, Stroobant V, Vertommen D, Vincent MF, Bommer GT, Veiga-da-Cunha M, Van Schaftingen E (Dec 2011). "Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading". The Journal of Biological Chemistry. 286 (50): 42992–3003. doi:10.1074/jbc.m111.281527. PMC 3234807. PMID 22016388.


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