Results 161 to 170 of about 5,767 (207)

Functional characterization of rat glutaryl-CoA dehydrogenase and its comparison with straight-chain acyl-CoA dehydrogenase

Bioorganic & Medicinal Chemistry Letters, 2011
Glutaryl-CoA dehydrogenase catalyzes the oxidative decarboxylation of the γ-carboxylate of the substrate, glutaryl-CoA, to yield crotonyl-CoA and CO(2). The enzyme is a member of the acyl-CoA dehydrogenase (ACD) family of flavoproteins. In the present study, the catalytic properties of this enzyme, including its substrate specificity, isomerase ...
Long, Wu, Yuqin, Qiao, Jinbo, Gao, Guisheng, Deng, Wenhua, Yu, Gong, Chen, Ding, Li   +6 more
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Kinetic Mechanism of Glutaryl-CoA Dehydrogenase

Biochemistry, 2006
Glutaryl-CoA dehydrogenase (GCD) is a homotetrameric enzyme containing one noncovalently bound FAD per monomer that oxidatively decarboxylates glutaryl-CoA to crotonyl-CoA and CO2. GCD belongs to the family of acyl-CoA dehydrogenases that are evolutionarily conserved in their sequence, structure, and function.
K Sudhindra, Rao, Mark, Albro, Timothy M, Dwyer, Frank E, Frerman   +3 more
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Mechanism of action of glutaryl-CoA and butyryl-CoA dehydrogenases. Purification of glutaryl-CoA dehydrogenase

Biochemistry, 1981
Glutaryl-CoA dehydrogenase, a flavoprotein, catalyzes the reaction -OOCCH3CH2--CH2COSR (FAD leads to FADH2) leads to CH3CH = CHCOSR + CO2 (SR = CoA or pantetheine). With the isolated enzyme, a dye serves as the final electron acceptor. The enzyme from Pseudomonas fluorescens (ATCC 11250) has been purified to homogeneity.
B, Gomes, G, Fendrich, R H, Abeles
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Emergency treatment in glutaryl‐CoA dehydrogenase deficiency

Journal of Inherited Metabolic Disease, 2004
AbstractSummary: The history of glutaryl‐CoA dehydrogenase deficiency is determined by acute encephalopathic crises that are precipitated by common febrile diseases, vaccinations or surgical interventions during infancy and early childhood. Such crises result in an irreversible destruction of the basal ganglia (in particular of the putamina), and ...
S, Kölker, C R, Greenberg, M, Lindner, E, Müller, E R, Naughten, G F, Hoffmann   +5 more
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Neonatal screening for glutaryl‐CoA dehydrogenase deficiency

Journal of Inherited Metabolic Disease, 2004
AbstractSummary: Acute encephalopathic crisis in glutaryl‐CoA dehydrogenase deficiency results in an unfavourable disease course and poor outcome, dominated by dystonia, feeding problems, seizures and secondary complications, and quite often leading to early death.
M, Lindner, S, Kölker, A, Schulze, E, Christensen, C R, Greenberg, G F, Hoffmann   +5 more
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Maintenance treatment of glutaryl‐CoA dehydrogenase deficiency

Journal of Inherited Metabolic Disease, 2004
AbstractSummary: This paper summarizes the published experience as well as results of the 3rd International Workshop on Glutaryl‐CoA Dehydrogenase Deficiency held in October 2003 in Heidelberg, Germany, on the topic treatment of patients with glutaryl‐CoA dehydrogenase (GCDH) deficiency.
C, Mühlhausen, G F, Hoffmann, K A, Strauss, S, Kölker, J G, Okun, C R, Greenberg, E R, Naughten, K, Ullrich   +7 more
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Animal models for glutaryl‐CoA dehydrogenase deficiency

Journal of Inherited Metabolic Disease, 2004
AbstractSummary:In vitro studies suggest that excitotoxic cell damage is an underlying mechanism for the acute striatal damage in glutaryl‐CoA dehydrogenase (GCDH) deficiency. It is believed to result from an imbalance of glutamatergic and GABAergic neurotransmission induced by the accumulating organic acids 3‐hydroxyglutaric acid (3‐OH‐GA) and to a ...
D M, Koeller, S, Sauer, M, Wajner, C F, de Mello, S I, Goodman, M, Woontner, C, Mühlhausen, J G, Okun, S, Kölker   +8 more
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Excitotoxicity and bioenergetics in glutaryl‐CoA dehydrogenase deficiency

Journal of Inherited Metabolic Disease, 2004
AbstractSummary: Glutaryl‐CoA dehydrogenase deficiency is an inherited organic acid disorder with predominantly neurological presentation. The biochemical hallmark of this disease is an accumulation and enhanced urinary excretion of two key organic acids, glutaric acid and 3‐hydroxyglutaric acid.
S, Kölker, D M, Koeller, S, Sauer, F, Hörster, M A, Schwab, G F, Hoffmann, K, Ullrich, J G, Okun   +7 more
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Pathomechanisms of neurodegeneration in glutaryl‐CoA dehydrogenase deficiency

Annals of Neurology, 2003
AbstractGlutaryl‐CoA dehydrogenase deficiency is an inherited organic aciduria with predominantly neurological presentation. Biochemically, it is characterized by an accumulation and enhanced urinary excretion of two key organic acids, glutaric acid and 3‐hydroxyglutaric acid.
Stefan, Kölker, David M, Koeller, Jürgen G, Okun, Georg F, Hoffmann   +3 more
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Late-onset neurologic disease in glutaryl-CoA dehydrogenase deficiency

Neurology, 2005
Neurologic disease in glutaryl-CoA dehydrogenase (GCDH) deficiency usually presents with acute encephalopathic crises before 2 years of age. The authors report two previously asymptomatic patients with macrocephaly presenting with progressive neurologic deterioration and a severe leukoencephalopathy during adolescence or adulthood.
S, Külkens, I, Harting, S, Sauer, J, Zschocke, G F, Hoffmann, S, Gruber, O A, Bodamer, S, Kölker   +7 more
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