acyl-CoA dehydrogenase

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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).
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TMS tests: Phenylketonuria, Isovaleric Acidemia, Type 1 Glutaric Aciduria, Medium Chain Acyl CoA Dehydrogenase (MCAD) deficiency, HMG CoA lyase deficiency.
Newborn screening program for Oman: the time is here and now
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.
Tamizaje neonatal por espectrometria de masas en tandem: actualizacion
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.
'Lifesaving' test for all
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.
Common causes of complex disorders
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.
Sam's Story
Diagnosis of medium chain acyl CoA dehydrogenase deficiency by measurement of cis-4-decenoic acid in dried blood spots.
Rapid diagnosis of MCAD deficiency: quantitative analysis of octanoylcarnitine and other acylcarnitines in newborn blood spots by tandem mass spectrometry
Mitochondrial oxidation of several amino acids and fatty acids involves acyl CoA intermediates, which are oxidized by flavin-containing acyl CoA dehydrogenases.
Defect in dimethylglycine dehydrogenase, a new inborn error of metabolism: NMR spectroscopy study

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