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Literature summary for 1.3.8.6 extracted from

  • Westover, J.B.; Goodman, S.I.; Frerman, F.E.
    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.
    View publication on PubMed

Cloned(Commentary)

Cloned (Comment) Organism
expression of wild-type and mutant enzymes in Escherichia coli Homo sapiens

Protein Variants

Protein Variants Comment Organism
A421T site-directed mutagenesis, altered Km, kcat is only slightly affected, slightly reduced activity compared to the wild-type enzyme Homo sapiens
A421V site-directed mutagenesis, altered Km, kcat is only slightly affected, reduced activity compared to the wild-type enzyme Homo sapiens
A433E site-directed mutagenesis, nearly inactive mutant Homo sapiens
A433V site-directed mutagenesis, altered Km, kcat is only slightly affected, reduced activity compared to the wild-type enzyme Homo sapiens
T429M site-directed mutagenesis, altered Km, kcat is only slightly affected, reduced activity compared to the wild-type enzyme Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
additional information
-
additional information steady-state kinetics Homo sapiens
0.0002
-
electron transfer flavoprotein mutant T429M, pH 7.6, 25°C Homo sapiens
0.0004
-
electron transfer flavoprotein mutant A433V, pH 7.6, 25°C Homo sapiens
0.0004
-
electron transfer flavoprotein recombinant wild-type enzyme, pH 7.6, 25°C Homo sapiens
0.0005
-
electron transfer flavoprotein mutant A421T, pH 7.6, 25°C Homo sapiens
0.0011
-
electron transfer flavoprotein mutant A421V, pH 7.6, 25°C Homo sapiens
0.0064
-
glutaryl-CoA recombinant wild-type enzyme, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein Homo sapiens
0.0074
-
glutaryl-CoA mutant A421T, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein Homo sapiens
0.0123
-
glutaryl-CoA mutant A421V, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein Homo sapiens
0.0261
-
glutaryl-CoA mutant A433V, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein Homo sapiens
0.0481
-
glutaryl-CoA mutant T429M, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein Homo sapiens

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
additional information
-
-
Homo sapiens
43600
-
4 * 43600, recombinant wild-type enzyme, SDS-PAGE Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
glutaryl-CoA + electron transfer flavoprotein Homo sapiens
-
crotonoyl-CoA + CO2 + reduced electron transfer flavoprotein
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

Purification (Comment) Organism
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]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
0.00036
-
recombinant mutant T429M, crude enzyme extract Homo sapiens
0.00086
-
recombinant mutant A421V, crude enzyme extract Homo sapiens
0.00151
-
recombinant mutant A321T, crude enzyme extract Homo sapiens
0.00174
-
recombinant wild-type enzyme, crude enzyme extract Homo sapiens
0.0097
-
recombinant mutant A433V, crude enzyme extract Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
glutaryl-CoA + 2,6-dichlorophenol indophenol
-
Homo sapiens crotonoyl-CoA + CO2 + reduced 2,6-dichlorophenol indophenol
-
?
glutaryl-CoA + electron transfer flavoprotein
-
Homo sapiens crotonoyl-CoA + CO2 + reduced electron transfer flavoprotein
-
?

Subunits

Subunits Comment Organism
More analysis of tertiary structure of wild-type and mutant enzymes, overview Homo sapiens
tetramer 4 * 43600, recombinant wild-type enzyme, SDS-PAGE Homo sapiens

Synonyms

Synonyms Comment Organism
GCD
-
Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
25
-
assay at Homo sapiens

Temperature Stability [°C]

Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
51.9
-
denaturation of mutant A421V, pH 7.0 Homo sapiens
52
-
denaturation of mutant A433V, pH 7.0 Homo sapiens
53.7
-
denaturation of mutant T429M, pH 7.0 Homo sapiens
55.7
-
denaturation of mutant A421T, pH 7.0 Homo sapiens
63.8
-
denaturation of recombinant wild-type enzyme, pH 7.0 Homo sapiens

Turnover Number [1/s]

Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
4.6
-
glutaryl-CoA mutant A421V, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein Homo sapiens
4.8
-
glutaryl-CoA mutant A421T, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein Homo sapiens
4.9
-
glutaryl-CoA mutant A433V, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein Homo sapiens
6.7
-
glutaryl-CoA recombinant wild-type enzyme, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein Homo sapiens
6.9
-
glutaryl-CoA mutant T429M, pH 7.6, 25°C, electron acceptor is electron transfer flavoprotein Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7.6
-
assay at, electron acceptor electron transfer flavoprotein Homo sapiens
8
-
assay at, electron acceptor 2,6-dichlorophenol indophenol Homo sapiens

Cofactor

Cofactor Comment Organism Structure
FAD prosthetic group, 1 per subunit Homo sapiens