acyl-CoA dehydrogenase

(redirected from Acyl CoA dehydrogenase)
Also found in: Wikipedia.

acyl-CoA dehydrogenase

/ac·yl-CoA de·hy·dro·gen·ase/ (de-hi´dro-jen-ās) any of several enzymes that catalyze the oxidation of acyl coenzyme A thioesters as a step in the degradation of fatty acids. Individual enzymes are specific for certain ranges of acyl chain lengths: long-chain a.-CoA d. (LCAD), medium-chain a.-CoA d. (MCAD), and short-chain a.-CoA d. (SCAD).
References in periodicals archive ?
TMS tests: Phenylketonuria, Isovaleric Acidemia, Type 1 Glutaric Aciduria, Medium Chain Acyl CoA Dehydrogenase (MCAD) deficiency, HMG CoA lyase deficiency.
Predictive value, clinical status and genotype of medium-chain acyl CoA dehydrogenase deficiency (MCADD) ascertained by screening at one week of age using electrospray tandem mass spectrometry of underivatized blood spots: findings from a UK multicentre trial.
Medium Chain Acyl CoA Dehydrogenase Deficiency (MCADD) can be easily managed once diagnosed but often the first sign is when the sufferer is taken seriously ill.
Well-known mitochondrial diseases of children are Leigh's syndrome, cardiomyopathty, and medium-chain acyl CoA dehydrogenase (MCAD) deficiency Typical mitochondrial diseases of young adults include Leber's hereditary optic neuropathy and Kearns-Sayre syndrome.
Many disorders and syndromes were eliminated as the focus turned to something called short-chain acyl CoA dehydrogenase deficiency, a fatty acid oxidation disorder within the larger category of mitochondrial disorders.
Diagnosis of medium chain acyl CoA dehydrogenase deficiency by measurement of cis-4-decenoic acid in dried blood spots.
Mitochondrial oxidation of several amino acids and fatty acids involves acyl CoA intermediates, which are oxidized by flavin-containing acyl CoA dehydrogenases.