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EC Tree
IUBMB Comments The enzyme, which exists in all vertebrates, decarboxylates ethylmalonyl-CoA, a potentially toxic compound that is formed in low amounts by the activity of EC 6.4.1.2, acetyl-CoA carboxylase and EC 6.4.1.3, propanoyl-CoA carboxylase. It prefers the S isomer, and can decarboxylate (R)-ethylmalonyl-CoA with lower efficiency. cf. EC 7.2.4.1, (S)-methylmalonyl-CoA decarboxylase (sodium-transporting).
The enzyme appears in viruses and cellular organisms
Synonyms
echdc1, ethylmalonyl-coa decarboxylase,
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ethylmalonyl-CoA decarboxylase
ECHDC1
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ethylmalonyl-CoA decarboxylase
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ethylmalonyl-CoA decarboxylase
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ethylmalonyl-CoA decarboxylase
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(S)-ethylmalonyl-CoA = butanoyl-CoA + CO2
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(S)-ethylmalonyl-CoA carboxy-lyase (butanoyl-CoA-forming)
The enzyme, which exists in all vertebrates, decarboxylates ethylmalonyl-CoA, a potentially toxic compound that is formed in low amounts by the activity of EC 6.4.1.2, acetyl-CoA carboxylase and EC 6.4.1.3, propanoyl-CoA carboxylase. It prefers the S isomer, and can decarboxylate (R)-ethylmalonyl-CoA with lower efficiency. cf. EC 7.2.4.1, (S)-methylmalonyl-CoA decarboxylase (sodium-transporting).
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(S)-ethylmalonyl-CoA
butanoyl-CoA + CO2
methylmalonyl-CoA
propanoyl-CoA + CO2
additional information
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(S)-ethylmalonyl-CoA
butanoyl-CoA + CO2
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best substrate, it is likely that the enzyme catalyzes preferentially the decarboxylation of one of the two isomers
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(S)-ethylmalonyl-CoA
butanoyl-CoA + CO2
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best substrate, it is likely that the enzyme catalyzes preferentially the decarboxylation of one of the two isomers. Recombinant ethylmalonyl-CoA decarboxylase acts preferentially on (S)-ethylmalonyl-CoA
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(S)-ethylmalonyl-CoA
butanoyl-CoA + CO2
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best substrate, it is likely that the enzyme catalyzes preferentially the decarboxylation of one of the two isomers
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methylmalonyl-CoA
propanoyl-CoA + CO2
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lower activity
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methylmalonyl-CoA
propanoyl-CoA + CO2
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lower activity
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methylmalonyl-CoA
propanoyl-CoA + CO2
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lower activity
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additional information
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the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity
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additional information
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the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity
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additional information
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the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity
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MgATP2-
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inhibits the enzyme slightly
additional information
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ECHDC1-specific siRNAs decrease the ethylmalonyl-CoA decarboxylase activity in human cells and increase the formation of ethylmalonate, most particularly in cells incubated with butyrate
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Breast Neoplasms
Genome-wide association study provides evidence for a breast cancer risk locus at 6q22.33.
Breast Neoplasms
Multi-modal meta-analysis of cancer cell line omics profiles identifies ECHDC1 as a novel breast tumor suppressor.
Breast Neoplasms
The RNF146 and ECHDC1 genes as candidates for inherited breast and ovarian cancer in Jewish Ashkenazi women.
ethylmalonyl-coa decarboxylase deficiency
ECHDC1 knock-out mice accumulate ethyl-branched lipids and excrete abnormal intermediates of branched-chain fatty acid metabolism.
Neoplasms
Multi-modal meta-analysis of cancer cell line omics profiles identifies ECHDC1 as a novel breast tumor suppressor.
Neoplasms
The RNF146 and ECHDC1 genes as candidates for inherited breast and ovarian cancer in Jewish Ashkenazi women.
Ovarian Neoplasms
The RNF146 and ECHDC1 genes as candidates for inherited breast and ovarian cancer in Jewish Ashkenazi women.
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0.00096 - 0.0065
(S)-ethylmalonyl-CoA
0.0031 - 0.0151
methylmalonyl-CoA
0.00096
(S)-ethylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
0.0065
(S)-ethylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
0.0031
methylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
0.0151
methylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
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9.5 - 10
(S)-ethylmalonyl-CoA
1.61 - 1.81
methylmalonyl-CoA
9.5
(S)-ethylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
10
(S)-ethylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
1.61
methylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
1.81
methylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
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1500 - 9900
(S)-ethylmalonyl-CoA
110 - 510
methylmalonyl-CoA
1500
(S)-ethylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
9900
(S)-ethylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
110
methylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
510
methylmalonyl-CoA
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pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
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8800
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purified recombinant ECHDC1, pH 7.1, 30°C
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7.1
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assay at
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30
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assay at
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brenda
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UniProt
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UniProt
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additional information
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subcellular localization analysis, overview
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malfunction
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enzyme mutation may be involved in the development of certain forms of ethylmalonic aciduria
metabolism
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ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase
additional information
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ECHDC1-specific siRNAs decrease the ethylmalonyl-CoA decarboxylase activity in human cells and increase the formation of ethylmalonate, most particularly in cells incubated with butyrate
physiological function
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ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase
physiological function
ECHDC1 expression is increased in gemcitabine-resistant bladder cancer cells, and is involved in their cell growth. Silencing of ECHDC1 significantly inhibits the proliferation of a gemcitabine-resistant UMUC-3-derived cell line. Silencing of ECHDC1 induces upregulation of p27, which is critical for cell cycle arrest in the G1 phase, and induces G1 arrest
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ECHD1_BOVIN
306
0
33542
Swiss-Prot
other Location (Reliability: 2 )
ECHD1_CHICK
298
0
32517
Swiss-Prot
Mitochondrion (Reliability: 2 )
ECHD1_DANRE
302
0
32326
Swiss-Prot
Mitochondrion (Reliability: 2 )
ECHD1_HUMAN
307
0
33698
Swiss-Prot
other Location (Reliability: 4 )
ECHD1_MOUSE
322
0
35467
Swiss-Prot
other Location (Reliability: 2 )
ECHD1_PONAB
301
0
32985
Swiss-Prot
other Location (Reliability: 5 )
ECHD1_RAT
299
0
32631
Swiss-Prot
other Location (Reliability: 5 )
ECHD1_XENLA
299
0
32656
Swiss-Prot
Mitochondrion (Reliability: 2 )
ECHD1_XENTR
299
0
32907
Swiss-Prot
Mitochondrion (Reliability: 2 )
A0A812C146_SEPPH
154
0
17763
TrEMBL
other Location (Reliability: 2 )
A0A8B6BQW5_MYTGA
293
0
32071
TrEMBL
Mitochondrion (Reliability: 3 )
A0A8B6GPV9_MYTGA
285
0
30809
TrEMBL
other Location (Reliability: 1 )
A0A6J8BS25_MYTCO
292
0
32218
TrEMBL
Mitochondrion (Reliability: 2 )
A0A8M1N645_DANRE
165
0
18049
TrEMBL
Mitochondrion (Reliability: 1 )
A0A8M1P9L1_DANRE
302
0
32360
TrEMBL
Mitochondrion (Reliability: 1 )
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34000
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x * 34000, recombinant ECHDC1, SDS-PAGE
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x * 34000, recombinant ECHDC1, SDS-PAGE
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synthesis
improved artificial pathway for the biosynthesis of poly((R)-3-hydroxybutyrate-co-(R)-3-hydroxyhexanoate) with high 3-hydroxyhexanoate composition from fructose in Ralstonia eutropha. Introduction of EchDC1, encoding codon-optimized ethylmalonyl-CoA decarboxylase increases poly((R)-3-hydroxybutyrate-co-(R)-3-hydroxyhexanoate) biosynthesis, probably by converting ethylmalonyl-CoA generated by the reductive carboxylase activity of crotonyl-CoA carboxylase/reductase back into butyryl-CoA
additional information
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expression of siRNA in HEK-293T cells suppresses the enzyme with an about 3fold decrease in ECHDC1 mRNA levels
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native enzyme from liver by anion exchange chromatography
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recombinant enzyme by metal affinity chromatography
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ECHDC1 from mouse brain cDNA, expression as N-terminally His-tagged enzyme
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medicine
ECHDC1 expression is increased in gemcitabine-resistant bladder cancer cells, and is involved in their cell growth. Silencing of ECHDC1 significantly inhibits the proliferation of a gemcitabine-resistant UMUC-3-derived cell line. Silencing of ECHDC1 induces upregulation of p27, which is critical for cell cycle arrest in the G1 phase, and induces G1 arrest
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Linster, C.L.; Noel, G.; Stroobant, V.; Vertommen, D.; Vincent, M.F.; Bommer, G.T.; Veiga-da-Cunha, M.; Van Schaftingen, E.
Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading
J. Biol. Chem.
286
42992-43003
2011
Homo sapiens, Mus musculus, Rattus norvegicus
brenda
Insomphun, C.; Xie, H.; Mifune, J.; Kawashima, Y.; Orita, I.; Nakamura, S.; Fukui, T.
Improved artificial pathway for biosynthesis of poly(3-hydroxybutyrate-co-3-hydroxyhexanoate) with high C6-monomer composition from fructose in Ralstonia eutropha
Metab. Eng.
27
38-45
2015
Mus musculus (Q9D9V3), Mus musculus
brenda
Asai, S.; Miura, N.; Sawada, Y.; Noda, T.; Kikugawa, T.; Tanji, N.; Saika, T.
Silencing of ECHDC1 inhibits growth of gemcitabine-resistant bladder cancer cells
Oncol. Lett.
15
522-527
2018
Homo sapiens (Q9NTX5), Homo sapiens
brenda
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4.1.1.94 ethylmalonyl-CoA decarboxylase
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