We're sorry, but BRENDA doesn't work properly without JavaScript. Please make sure you have JavaScript enabled in your browser settings.
Information on EC 4.1.1.94 - ethylmalonyl-CoA decarboxylase for references in articles please use BRENDA:EC4.1.1.94Word Map on EC 4.1.1.94
Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
Specify your search results
The enzyme appears in viruses and cellular organisms
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
ethylmalonyl-CoA decarboxylase
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
(S)-ethylmalonyl-CoA = butanoyl-CoA + CO2
-
-
-
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
(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)-methylmalonyl-CoA with lower efficiency. cf. EC 4.1.1.41 (methylmalonyl-CoA decarboxylase).
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
ethylmalonyl-CoA decarboxylase
ECHDC1
-
-
ethylmalonyl-CoA decarboxylase
-
-
ethylmalonyl-CoA decarboxylase
-
-
ethylmalonyl-CoA decarboxylase
-
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
-
-
-
brenda
-
-
-
brenda
-
-
-
brenda
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
malfunction
-
enzyme mutation may be involved in the development of certain forms of ethylmalonic aciduria
metabolism
-
ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase
physiological function
-
ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase
additional information
-
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
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
(S)-ethylmalonyl-CoA
butanoyl-CoA + CO2
methylmalonyl-CoA
propanoyl-CoA + CO2
additional information
?
-
(S)-ethylmalonyl-CoA
butanoyl-CoA + CO2
-
best substrate, it is likely that the enzyme catalyzes preferentially the decarboxylation of one of the two isomers
-
-
?
(S)-ethylmalonyl-CoA
butanoyl-CoA + CO2
-
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
-
-
?
(S)-ethylmalonyl-CoA
butanoyl-CoA + CO2
-
best substrate, it is likely that the enzyme catalyzes preferentially the decarboxylation of one of the two isomers
-
-
?
methylmalonyl-CoA
propanoyl-CoA + CO2
-
lower activity
-
-
?
methylmalonyl-CoA
propanoyl-CoA + CO2
-
lower activity
-
-
?
methylmalonyl-CoA
propanoyl-CoA + CO2
-
lower activity
-
-
?
additional information
?
-
-
the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity
-
-
-
additional information
?
-
-
the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity
-
-
-
additional information
?
-
-
the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity
-
-
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
MgATP2-
-
inhibits the enzyme slightly
additional information
-
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
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
0.00096 - 0.0065
(S)-ethylmalonyl-CoA
0.0031 - 0.0151
methylmalonyl-CoA
0.00096
(S)-ethylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
0.0065
(S)-ethylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
0.0031
methylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
0.0151
methylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
9.5 - 10
(S)-ethylmalonyl-CoA
1.61 - 1.81
methylmalonyl-CoA
9.5
(S)-ethylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
10
(S)-ethylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
1.61
methylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
1.81
methylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
1500 - 9900
(S)-ethylmalonyl-CoA
110 - 510
methylmalonyl-CoA
1500
(S)-ethylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
9900
(S)-ethylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
110
methylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2-
510
methylmalonyl-CoA
-
pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
8800
-
purified recombinant ECHDC1, pH 7.1, 30°C
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
7.1
-
assay at
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
30
-
assay at
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
-
-
brenda
-
-
brenda
-
-
brenda
-
-
brenda
-
-
brenda
-
-
brenda
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
additional information
-
subcellular localization analysis, overview
-
brenda
-
-
brenda
-
-
brenda
-
-
brenda
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
34000
-
x * 34000, recombinant ECHDC1, SDS-PAGE
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
?
-
x * 34000, recombinant ECHDC1, SDS-PAGE
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
native enzyme from liver by anion exchange chromatography
-
recombinant enzyme by metal affinity chromatography
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
ECHDC1 from mouse brain cDNA, expression as N-terminally His-tagged enzyme
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
additional information
-
expression of siRNA in HEK-293T cells suppresses the enzyme with an about 3fold decrease in ECHDC1 mRNA levels
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
ECHD1_BOVIN
306
33542
Swiss-Prot
ECHD1_CHICK
298
32517
Swiss-Prot
ECHD1_DANRE
302
32326
Swiss-Prot
ECHD1_HUMAN
307
33698
Swiss-Prot
ECHD1_MOUSE
322
35467
Swiss-Prot
ECHD1_PONAB
301
32985
Swiss-Prot
ECHD1_RAT
299
32631
Swiss-Prot
ECHD1_XENLA
299
32656
Swiss-Prot
ECHD1_XENTR
299
32907
Swiss-Prot
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Breast Neoplasms
Genome-wide association study provides evidence for a breast cancer risk locus at 6q22.33.
Breast Neoplasms
The RNF146 and ECHDC1 genes as candidates for inherited breast and ovarian cancer in Jewish Ashkenazi women.
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.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
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
html completed