Crystallization (Comment) | Organism |
---|---|
15 mg/ml purified recombinant enzyme in 20 mM HEPES, pH 7.5, 100 mM NaCl, enzyme is free or in complex with 4-nitrobutyryl-CoA, hanging drop vapour diffusion method, equal volumes of protein and precipitant solution, the latter containing 100 mM MES, pH 6.5, 30% PEG monomethyl ether 5000, 0.2 M ammonium sulfate, 19°C, addition of 0.1% octyl beta-D-glucopyranoside, 0.4 mM acetoacetyl-CoA, with or without addition of 0.4 mM 4-nitrobutyryl-CoA, X-ray diffraction structure determination and analysis at 2.1 A and 2.6 A resolution, respectively | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
A377T | naturally occurring mutation from patient with glutaric acidemia type I, dissociation to inactive monomers or dimers | Homo sapiens |
A377V | naturally occurring mutation from patient with glutaric acidemia type I, dissociation to inactive monomers or dimers | Homo sapiens |
A389E | naturally occurring mutation from patient with glutaric acidemia type I, dissociation to inactive monomers or dimers | Homo sapiens |
A389V | naturally occurring mutation from patient with glutaric acidemia type I, dissociation to inactive monomers or dimers | Homo sapiens |
T385M | naturally occurring mutation from patient with glutaric acidemia type I, dissociation to inactive monomers or dimers | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Homo sapiens | 5739 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
glutaryl-CoA + electron transfer protein | Homo sapiens | - |
crotonoyl-CoA + CO2 + reduced electron transfer protein | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q92947 | mature mitochondrial enzyme without targeting sequence | - |
Reaction | Comment | Organism | Reaction ID |
---|---|---|---|
glutaryl-CoA + electron-transfer flavoprotein = crotonyl-CoA + CO2 + reduced electron-transfer flavoprotein | structural bases and mechanism of dehydrogenation and decarboxylation reactions, active site structure with and without bound substrate, Glu370 is important | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-nitrobutyryl-CoA + electron transfer protein | - |
Homo sapiens | ? + CO2 + reduced electron transfer protein | - |
? | |
glutaryl-CoA + electron transfer protein | - |
Homo sapiens | crotonoyl-CoA + CO2 + reduced electron transfer protein | - |
? |
Subunits | Comment | Organism |
---|---|---|
More | subunit interaction analysis of wild-type and mutant enzymes, overview | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
GCD | - |
Homo sapiens |
More | enzyme belongs to the acyl-CoA dehydrogenase family of enzymes | Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
FAD | flavoprotein | Homo sapiens |