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Literature summary 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)

EC Number Crystallization (Comment) Organism
1.3.8.6 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

EC Number Protein Variants Comment Organism
1.3.8.6 A377T naturally occurring mutation from patient with glutaric acidemia type I, dissociation to inactive monomers or dimers Homo sapiens
1.3.8.6 A377V naturally occurring mutation from patient with glutaric acidemia type I, dissociation to inactive monomers or dimers Homo sapiens
1.3.8.6 A389E naturally occurring mutation from patient with glutaric acidemia type I, dissociation to inactive monomers or dimers Homo sapiens
1.3.8.6 A389V naturally occurring mutation from patient with glutaric acidemia type I, dissociation to inactive monomers or dimers Homo sapiens
1.3.8.6 T385M naturally occurring mutation from patient with glutaric acidemia type I, dissociation to inactive monomers or dimers Homo sapiens

Localization

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

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 protein Homo sapiens
-
crotonoyl-CoA + CO2 + reduced electron transfer protein
-
?

Organism

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

Reaction

EC Number Reaction Comment Organism Reaction ID
1.3.8.6 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)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.3.8.6 4-nitrobutyryl-CoA + electron transfer protein
-
Homo sapiens ? + CO2 + reduced electron transfer protein
-
?
1.3.8.6 glutaryl-CoA + electron transfer protein
-
Homo sapiens crotonoyl-CoA + CO2 + reduced electron transfer protein
-
?

Subunits

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

Synonyms

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

Cofactor

EC Number Cofactor Comment Organism Structure
1.3.8.6 FAD flavoprotein Homo sapiens