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Literature summary extracted from

  • 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 (2011), J. Biol. Chem., 286, 42992-43003.
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

EC Number Cloned (Comment) Organism
4.1.1.94 ECHDC1 from mouse brain cDNA, expression as N-terminally His-tagged enzyme Mus musculus

Protein Variants

EC Number Protein Variants Comment Organism
4.1.1.94 additional information expression of siRNA in HEK-293T cells suppresses the enzyme with an about 3fold decrease in ECHDC1 mRNA levels Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
4.1.1.94 MgATP2- inhibits the enzyme slightly Mus musculus
4.1.1.94 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 Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
4.1.1.94 0.00096
-
 (S)-ethylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- Mus musculus
4.1.1.94 0.0031
-
 methylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- Mus musculus
4.1.1.94 0.0065
-
 (S)-ethylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- Mus musculus
4.1.1.94 0.0151
-
 methylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- Mus musculus

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
4.1.1.94 cytosol
-
 Mus musculus 5829
-
4.1.1.94 cytosol
-
 Homo sapiens 5829
-
4.1.1.94 cytosol
-
 Rattus norvegicus 5829
-
4.1.1.94 additional information subcellular localization analysis, overview Rattus norvegicus
-
-

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
4.1.1.94 34000
-
 x * 34000, recombinant ECHDC1, SDS-PAGE Mus musculus

Organism

EC Number Organism UniProt Comment Textmining
4.1.1.94 Homo sapiens
-
-
-
4.1.1.94 Mus musculus
-
-
-
4.1.1.94 Rattus norvegicus
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
4.1.1.94 native enzyme from liver by anion exchange chromatography Rattus norvegicus
4.1.1.94 recombinant enzyme by metal affinity chromatography Mus musculus

Source Tissue

EC Number Source Tissue Comment Organism Textmining
4.1.1.94 brown adipose tissue
-
 Mus musculus
-
4.1.1.94 HEK-293T cell
-
 Homo sapiens
-
4.1.1.94 kidney
-
 Mus musculus
-
4.1.1.94 liver
-
 Mus musculus
-
4.1.1.94 liver
-
 Homo sapiens
-
4.1.1.94 liver
-
 Rattus norvegicus
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
4.1.1.94 8800
-
 purified recombinant ECHDC1, pH 7.1, 30°C Mus musculus

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4.1.1.94 (S)-ethylmalonyl-CoA best substrate, it is likely that the enzyme catalyzes preferentially the decarboxylation of one of the two isomers Homo sapiens butanoyl-CoA + CO2
-
 ?
4.1.1.94 (S)-ethylmalonyl-CoA best substrate, it is likely that the enzyme catalyzes preferentially the decarboxylation of one of the two isomers Rattus norvegicus butanoyl-CoA + CO2
-
 ?
4.1.1.94 (S)-ethylmalonyl-CoA 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 Mus musculus butanoyl-CoA + CO2
-
 ?
4.1.1.94 methylmalonyl-CoA lower activity Mus musculus propanoyl-CoA + CO2
-
 ?
4.1.1.94 methylmalonyl-CoA lower activity Homo sapiens propanoyl-CoA + CO2
-
 ?
4.1.1.94 methylmalonyl-CoA lower activity Rattus norvegicus propanoyl-CoA + CO2
-
 ?
4.1.1.94 additional information the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity Mus musculus ?
-
 ?
4.1.1.94 additional information the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity Homo sapiens ?
-
 ?
4.1.1.94 additional information the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity Rattus norvegicus ?
-
 ?

Subunits

EC Number Subunits Comment Organism
4.1.1.94 ? x * 34000, recombinant ECHDC1, SDS-PAGE Mus musculus

Synonyms

EC Number Synonyms Comment Organism
4.1.1.94 ECHDC1
-
 Mus musculus
4.1.1.94 ECHDC1
-
 Homo sapiens
4.1.1.94 ECHDC1
-
 Rattus norvegicus
4.1.1.94 ethylmalonyl-CoA decarboxylase
-
 Mus musculus
4.1.1.94 ethylmalonyl-CoA decarboxylase
-
 Homo sapiens
4.1.1.94 ethylmalonyl-CoA decarboxylase
-
 Rattus norvegicus

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
4.1.1.94 30
-
 assay at Mus musculus
4.1.1.94 30
-
 assay at Homo sapiens
4.1.1.94 30
-
 assay at Rattus norvegicus

Turnover Number [1/s]

EC Number Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
4.1.1.94 1.61
-
 methylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- Mus musculus
4.1.1.94 1.81
-
 methylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- Mus musculus
4.1.1.94 9.5
-
 (S)-ethylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- Mus musculus
4.1.1.94 10
-
 (S)-ethylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- Mus musculus

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
4.1.1.94 7.1
-
 assay at Mus musculus
4.1.1.94 7.1
-
 assay at Homo sapiens
4.1.1.94 7.1
-
 assay at Rattus norvegicus

General Information

EC Number General Information Comment Organism
4.1.1.94 malfunction enzyme mutation may be involved in the development of certain forms of ethylmalonic aciduria Homo sapiens
4.1.1.94 metabolism ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase Homo sapiens
4.1.1.94 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 Homo sapiens
4.1.1.94 physiological function ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase Homo sapiens

kcat/KM [mM/s]

EC Number kcat/KM Value [1/mMs-1] kcat/KM Value Maximum [1/mMs-1] Substrate Comment Organism Structure
4.1.1.94 110
-
 methylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- Mus musculus
4.1.1.94 510
-
 methylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- Mus musculus
4.1.1.94 1500
-
 (S)-ethylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- Mus musculus
4.1.1.94 9900
-
 (S)-ethylmalonyl-CoA pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- Mus musculus