Results 111 to 120 of about 3,031 (152)

Effects of Ritonavir, Lopinavir, and Alcohol on ABC Transporters and Secretion of Bile Acid and Bilirubin in Senescent Hepatocytes. [PDF]

Int J Mol Sci
Chen L, Duran E, Headrick D, Ji C.
europepmc   +1 more source

Lysine: Sources, Metabolism, Physiological Importance, and Use as a Supplement. [PDF]

Int J Mol Sci
Holeček M.
europepmc   +1 more source

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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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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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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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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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Riboflavin-responsive glutaryl CoA dehydrogenase deficiency

Molecular Genetics and Metabolism, 2006
We report here riboflavin responsiveness in a patient with glutaryl CoA dehydrogenase (GCDH) deficiency, compound heterozygous for the S139L and P248L mutations and with 20% residual GCDH enzyme activity in vitro. Our results suggest the mitochondrial GCDH homotetramer remains intact with one of these mutations associated with the binding site of the ...
Ronald A, Chalmers, Murray D, Bain, Johannes, Zschocke   +2 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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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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