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Literature summary for 4.1.1.94 extracted from
- Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading (2011), J. Biol. Chem., 286, 42992-43003.
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| ECHDC1 from mouse brain cDNA, expression as N-terminally His-tagged enzyme | Mus musculus |
Protein Variants
| Protein Variants | Comment | Organism |
|---|---|---|
| additional information | expression of siRNA in HEK-293T cells suppresses the enzyme with an about 3fold decrease in ECHDC1 mRNA levels | Homo sapiens |
Inhibitors
| Inhibitors | Comment | Organism | Structure |
|---|---|---|---|
| MgATP2- | inhibits the enzyme slightly | Mus musculus |  |
| 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]
| KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|
| 0.00096 | - |
(S)-ethylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- | Mus musculus | |
| 0.0031 | - |
methylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- | Mus musculus | |
| 0.0065 | - |
(S)-ethylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- | Mus musculus | |
| 0.0151 | - |
methylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- | Mus musculus | |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| cytosol | - |
Mus musculus | 5829 | - |
| cytosol | - |
Homo sapiens | 5829 | - |
| cytosol | - |
Rattus norvegicus | 5829 | - |
| additional information | subcellular localization analysis, overview | Rattus norvegicus | - |
- |
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 34000 | - |
x * 34000, recombinant ECHDC1, SDS-PAGE | Mus musculus |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
| Mus musculus | - |
- |
- |
| Rattus norvegicus | - |
- |
- |
Purification (Commentary)
| Purification (Comment) | Organism |
|---|---|
| native enzyme from liver by anion exchange chromatography | Rattus norvegicus |
| recombinant enzyme by metal affinity chromatography | Mus musculus |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| brown adipose tissue | - |
Mus musculus | - |
| HEK-293T cell | - |
Homo sapiens | - |
| kidney | - |
Mus musculus | - |
| liver | - |
Mus musculus | - |
| liver | - |
Homo sapiens | - |
| liver | - |
Rattus norvegicus | - |
Specific Activity [micromol/min/mg]
| Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|
| 8800 | - |
purified recombinant ECHDC1, pH 7.1, 30°C | Mus musculus |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| (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 | - |
? | |
| (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 | - |
? | |
| (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 | - |
? | |
| methylmalonyl-CoA | lower activity | Mus musculus | propanoyl-CoA + CO2 | - |
? | |
| methylmalonyl-CoA | lower activity | Homo sapiens | propanoyl-CoA + CO2 | - |
? | |
| methylmalonyl-CoA | lower activity | Rattus norvegicus | propanoyl-CoA + CO2 | - |
? | |
| additional information | the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity | Mus musculus | ? | - |
? | |
| additional information | the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity | Homo sapiens | ? | - |
? | |
| additional information | the enzyme shows no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity | Rattus norvegicus | ? | - |
? | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| ? | x * 34000, recombinant ECHDC1, SDS-PAGE | Mus musculus |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| ECHDC1 | - |
Mus musculus |
| ECHDC1 | - |
Homo sapiens |
| ECHDC1 | - |
Rattus norvegicus |
| ethylmalonyl-CoA decarboxylase | - |
Mus musculus |
| ethylmalonyl-CoA decarboxylase | - |
Homo sapiens |
| ethylmalonyl-CoA decarboxylase | - |
Rattus norvegicus |
Temperature Optimum [°C]
| Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
|---|---|---|---|
| 30 | - |
assay at | Mus musculus |
| 30 | - |
assay at | Homo sapiens |
| 30 | - |
assay at | Rattus norvegicus |
Turnover Number [1/s]
| Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|
| 1.61 | - |
methylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- | Mus musculus | |
| 1.81 | - |
methylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- | Mus musculus | |
| 9.5 | - |
(S)-ethylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- | Mus musculus | |
| 10 | - |
(S)-ethylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- | Mus musculus | |
pH Optimum
| pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|
| 7.1 | - |
assay at | Mus musculus |
| 7.1 | - |
assay at | Homo sapiens |
| 7.1 | - |
assay at | Rattus norvegicus |
General Information
| General Information | Comment | Organism |
|---|---|---|
| malfunction | enzyme mutation may be involved in the development of certain forms of ethylmalonic aciduria | Homo sapiens |
| metabolism | ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase | Homo sapiens |
| 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 |
| physiological function | ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase | Homo sapiens |
kcat/KM [mM/s]
| kcat/KM Value [1/mMs-1] | kcat/KM Value Maximum [1/mMs-1] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|
| 110 | - |
methylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- | Mus musculus | |
| 510 | - |
methylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- | Mus musculus | |
| 1500 | - |
(S)-ethylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in presence of 5 mM ATP-Mg2- | Mus musculus | |
| 9900 | - |
(S)-ethylmalonyl-CoA | pH 7.1, 30°C, recombinant enzyme in absence of ATP-Mg2- | Mus musculus | |
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Release 2023.1 (January 2023)
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