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Literature summary for 1.3.8.6 extracted from

  • Fu, Z.; Wang, M.; Paschke, R.; Rao, K.S.; Frerman, F.E.; Kim, J.J.
    Crystal structures of human glutaryl-CoA dehydrogenase with and without an alternate substrate: structural bases of dehydrogenation and decarboxylation reactions (2004), Biochemistry, 43, 9674-9684.
    View publication on PubMed

Crystallization (Commentary)

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

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

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

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

Organism UniProt Comment Textmining
Homo sapiens Q92947 mature mitochondrial enzyme without targeting sequence
-

Reaction

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 and Products (Substrate)

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

Subunits Comment Organism
More subunit interaction analysis of wild-type and mutant enzymes, overview Homo sapiens

Synonyms

Synonyms Comment Organism
GCD
-
Homo sapiens
More enzyme belongs to the acyl-CoA dehydrogenase family of enzymes Homo sapiens

Cofactor

Cofactor Comment Organism Structure
FAD flavoprotein Homo sapiens