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Literature summary extracted from
- Pathogenic mutations in the carboxyl-terminal domain of glutaryl-CoA dehydrogenase: effects on catalytic activity and the stability of the tetramer (2003), Mol. Genet. Metab., 79, 245-256.
Cloned(Commentary)
| EC Number | Cloned (Comment) | Organism |
|---|---|---|
| 1.3.8.6 | expression of wild-type and mutant enzymes in Escherichia coli | Homo sapiens |
Protein Variants
| EC Number | Protein Variants | Comment | Organism |
|---|---|---|---|
| 1.3.8.6 | A421T | site-directed mutagenesis, altered Km, kcat is only slightly affected, slightly reduced activity compared to the wild-type enzyme | Homo sapiens |
| 1.3.8.6 | A421V | site-directed mutagenesis, altered Km, kcat is only slightly affected, reduced activity compared to the wild-type enzyme | Homo sapiens |
| 1.3.8.6 | A433E | site-directed mutagenesis, nearly inactive mutant | Homo sapiens |
| 1.3.8.6 | A433V | site-directed mutagenesis, altered Km, kcat is only slightly affected, reduced activity compared to the wild-type enzyme | Homo sapiens |
| 1.3.8.6 | T429M | site-directed mutagenesis, altered Km, kcat is only slightly affected, reduced activity compared to the wild-type enzyme | Homo sapiens |
KM Value [mM]
| EC Number | KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|---|
| 1.3.8.6 | additional information | - |
additional information | steady-state kinetics | Homo sapiens | |
| 1.3.8.6 | 0.0002 | - |
electron transfer flavoprotein | mutant T429M, pH 7.6, 25°C | Homo sapiens |  |
| 1.3.8.6 | 0.0004 | - |
electron transfer flavoprotein | mutant A433V, pH 7.6, 25°C | Homo sapiens | |
| 1.3.8.6 | 0.0004 | - |
electron transfer flavoprotein | recombinant wild-type enzyme, pH 7.6, 25°C | Homo sapiens | |
| 1.3.8.6 | 0.0005 | - |
electron transfer flavoprotein | mutant A421T, pH 7.6, 25°C | Homo sapiens | |
| 1.3.8.6 | 0.0011 | - |
electron transfer flavoprotein | mutant A421V, pH 7.6, 25°C | Homo sapiens | |
| 1.3.8.6 | 0.0064 | - |
glutaryl-CoA | recombinant wild-type enzyme, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein | Homo sapiens | |
| 1.3.8.6 | 0.0074 | - |
glutaryl-CoA | mutant A421T, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein | Homo sapiens | |
| 1.3.8.6 | 0.0123 | - |
glutaryl-CoA | mutant A421V, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein | Homo sapiens | |
| 1.3.8.6 | 0.0261 | - |
glutaryl-CoA | mutant A433V, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein | Homo sapiens | |
| 1.3.8.6 | 0.0481 | - |
glutaryl-CoA | mutant T429M, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein | Homo sapiens | |
Molecular Weight [Da]
| EC Number | Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|---|
| 1.3.8.6 | additional information | - |
- |
Homo sapiens |
| 1.3.8.6 | 43600 | - |
4 * 43600, recombinant wild-type enzyme, SDS-PAGE | Homo sapiens |
Natural Substrates/ Products (Substrates)
| EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.3.8.6 | glutaryl-CoA + electron transfer flavoprotein | Homo sapiens | - |
crotonoyl-CoA + CO2 + reduced electron transfer flavoprotein | - |
? | |
Organism
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 1.3.8.6 | Homo sapiens | - |
- |
- |
Purification (Commentary)
| EC Number | Purification (Comment) | Organism |
|---|---|---|
| 1.3.8.6 | recombinant wild-type and mutant enzymes from Escherichia coli, except for mutant A433E, different yields from recombinant cell culture | Homo sapiens |
Specific Activity [micromol/min/mg]
| EC Number | Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|---|
| 1.3.8.6 | 0.00036 | - |
recombinant mutant T429M, crude enzyme extract | Homo sapiens |
| 1.3.8.6 | 0.00086 | - |
recombinant mutant A421V, crude enzyme extract | Homo sapiens |
| 1.3.8.6 | 0.00151 | - |
recombinant mutant A321T, crude enzyme extract | Homo sapiens |
| 1.3.8.6 | 0.00174 | - |
recombinant wild-type enzyme, crude enzyme extract | Homo sapiens |
| 1.3.8.6 | 0.0097 | - |
recombinant mutant A433V, crude enzyme extract | Homo sapiens |
Substrates and Products (Substrate)
| EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.3.8.6 | glutaryl-CoA + 2,6-dichlorophenol indophenol | - |
Homo sapiens | crotonoyl-CoA + CO2 + reduced 2,6-dichlorophenol indophenol | - |
? | |
| 1.3.8.6 | glutaryl-CoA + electron transfer flavoprotein | - |
Homo sapiens | crotonoyl-CoA + CO2 + reduced electron transfer flavoprotein | - |
? | |
Subunits
| EC Number | Subunits | Comment | Organism |
|---|---|---|---|
| 1.3.8.6 | More | analysis of tertiary structure of wild-type and mutant enzymes, overview | Homo sapiens |
| 1.3.8.6 | tetramer | 4 * 43600, recombinant wild-type enzyme, SDS-PAGE | Homo sapiens |
Synonyms
| EC Number | Synonyms | Comment | Organism |
|---|---|---|---|
| 1.3.8.6 | GCD | - |
Homo sapiens |
Temperature Optimum [°C]
| EC Number | Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
|---|---|---|---|---|
| 1.3.8.6 | 25 | - |
assay at | Homo sapiens |
Temperature Stability [°C]
| EC Number | Temperature Stability Minimum [°C] | Temperature Stability Maximum [°C] | Comment | Organism |
|---|---|---|---|---|
| 1.3.8.6 | 51.9 | - |
denaturation of mutant A421V, pH 7.0 | Homo sapiens |
| 1.3.8.6 | 52 | - |
denaturation of mutant A433V, pH 7.0 | Homo sapiens |
| 1.3.8.6 | 53.7 | - |
denaturation of mutant T429M, pH 7.0 | Homo sapiens |
| 1.3.8.6 | 55.7 | - |
denaturation of mutant A421T, pH 7.0 | Homo sapiens |
| 1.3.8.6 | 63.8 | - |
denaturation of recombinant wild-type enzyme, pH 7.0 | Homo sapiens |
Turnover Number [1/s]
| EC Number | Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|---|
| 1.3.8.6 | 4.6 | - |
glutaryl-CoA | mutant A421V, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein | Homo sapiens | |
| 1.3.8.6 | 4.8 | - |
glutaryl-CoA | mutant A421T, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein | Homo sapiens | |
| 1.3.8.6 | 4.9 | - |
glutaryl-CoA | mutant A433V, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein | Homo sapiens | |
| 1.3.8.6 | 6.7 | - |
glutaryl-CoA | recombinant wild-type enzyme, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein | Homo sapiens | |
| 1.3.8.6 | 6.9 | - |
glutaryl-CoA | mutant T429M, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein | Homo sapiens | |
pH Optimum
| EC Number | pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|---|
| 1.3.8.6 | 7.6 | - |
assay at, electron acceptor electron transfer flavoprotein | Homo sapiens |
| 1.3.8.6 | 8 | - |
assay at, electron acceptor 2,6-dichlorophenol indophenol | Homo sapiens |
Cofactor
| EC Number | Cofactor | Comment | Organism | Structure |
|---|---|---|---|---|
| 1.3.8.6 | FAD | prosthetic group, 1 per subunit | Homo sapiens | |
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