Ethylmalonyl-CoA decarboxylase

Ethylmalonyl-CoA decarboxylase
Ethylmalonyl-CoA decarboxylase
Identifiers
EC no.	4.1.1.94
Databases
IntEnz	IntEnz view
BRENDA	BRENDA entry
ExPASy	NiceZyme view
KEGG	KEGG entry
MetaCyc	metabolic pathway
PRIAM	profile
PDB structures	RCSB PDB PDBe PDBsum
PMC
Search
PMC	articles
PubMed	articles
NCBI	proteins

ECHDC1
Identifiers
Aliases	ECHDC1, HEL-S-76, MMCD, dJ351K20.2, ethylmalonyl-CoA decarboxylase 1
External IDs	OMIM: 612136 MGI: 1277169 HomoloGene: 23106 GeneCards: ECHDC1
EC number	7.2.4.3
Gene location (Human)
Chr.	Chromosome 6 (human)
End	127,343,609 bp
Gene location (Mouse)
Chr.	Chromosome 10 (mouse)
End	29,223,465 bp
RNA expression pattern
	Top expressed in
	retinal pigment epithelium; ; palpebral conjunctiva; ; parotid gland; ; vulva; ; lower lobe of lung; ; germinal epithelium; ; visceral pleura; ; monocyte; ; lactiferous duct; ; rectum;
	Top expressed in
	brown adipose tissue; ; spermatocyte; ; intercostal muscle; ; lip; ; interventricular septum; ; white adipose tissue; ; left lobe of liver; ; subcutaneous adipose tissue; ; parotid gland; ; proximal tubule;
	More reference expression data
	n/a
Gene ontology
Molecular function	methylmalonyl-CoA decarboxylase activity; catalytic activity; carboxy-lyase activity; lyase activity; enoyl-CoA hydratase activity;
Cellular component	cytoplasm; cytosol; mitochondrion;
Biological process	metabolism; fatty acid beta-oxidation;
	Sources:Amigo / QuickGO
Orthologs
	55862
	52665
	ENSG00000093144
	ENSMUSG00000019883
	Q9NTX5
	Q9D9V3
	NM_001002030; NM_001105544; NM_001105545; NM_001139510; NM_018479
	NM_001110195; NM_025855
	NP_001002030; NP_001099014; NP_001099015; NP_001132982; NP_060949
	NP_001103665; NP_080131
	Wikidata
View/Edit Human	View/Edit Mouse

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

\rightleftharpoons

butanoyl-CoA + CO₂

The vertebrate enzyme decarboxylates ethylmalonyl-CoA.

References

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000093144 – Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000019883 – Ensembl, May 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ 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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v t e Carbon–carbon lyases (EC 4.1)
4.1.1: Carboxy-lyases	Acetoacetate decarboxylase Adenosylmethionine decarboxylase Arginine decarboxylase Aromatic L-amino acid decarboxylase Glutamate decarboxylase Histidine decarboxylase Lysine decarboxylase Malonyl-CoA decarboxylase Ornithine decarboxylase Oxaloacetate decarboxylase Phosphoenolpyruvate carboxykinase Phosphoenolpyruvate carboxylase Phosphoribosylaminoimidazole carboxylase Pyrophosphomevalonate decarboxylase Pyruvate decarboxylase RuBisCO Uridine monophosphate synthetase/Orotidine 5'-phosphate decarboxylase Uroporphyrinogen III decarboxylase
4.1.2: Aldehyde-lyases	Fructose-bisphosphate aldolase Aldolase A Aldolase B Aldolase C 2-hydroxyphytanoyl-CoA lyase Threonine aldolase
4.1.3: Oxo-acid-lyases	Isocitrate lyase 3-hydroxy-3-methylglutaryl-CoA lyase
4.1.99: Other	Tryptophanase Photolyase CPD lyase Spore photoproduct lyase

v t e Enzymes
Activity	Active site Binding site Catalytic triad Oxyanion hole Enzyme promiscuity Diffusion-limited enzyme Cofactor Enzyme catalysis
Regulation	Allosteric regulation Cooperativity Enzyme inhibitor Enzyme activator
Classification	EC number Enzyme superfamily Enzyme family List of enzymes
Kinetics	Enzyme kinetics Eadie–Hofstee diagram Hanes–Woolf plot Lineweaver–Burk plot Michaelis–Menten kinetics
Types	EC1 Oxidoreductases (list) EC2 Transferases (list) EC3 Hydrolases (list) EC4 Lyases (list) EC5 Isomerases (list) EC6 Ligases (list) EC7 Translocases (list)