acyl-CoA dehydrogenases

acyl-CoA dehydrogenases

Enzymes that activate the first stage of the oxidation of fatty acids.
References in periodicals archive ?
In my own specialist area of lipid metabolism, deficiencies of the fatty acid metabolizing acyl-CoA dehydrogenases are defined as a group of diseases that may be potentially treatable with a combination of dietary manipulation and prevention of fasting, because these are essentially diseases of fasting intolerance.
Selective Inhibition of Acyl-CoA Dehydrogenases by a Metabolite of Hypoglycin.
Inactivation of General Acyl-CoA Dehydrogenase from Pig Kidney by a Metabolite of Hypoglycin A.
The first step of the [beta]-oxidation cycle is catalyzed by acyl-CoA dehydrogenases, whereas the enzymes of MTP catalyze the last 3 steps of [beta]-oxidation.
This is in contrast to the situation for human very long-chain, long-chain, and medium-chain acyl-CoA dehydrogenases, which are stereospecific for the S-enantiomers only of their methyl branched-chain substrates (24).
Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: iden tification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism.
As a newborn-screening assay, MS/MS is not merely a method for detecting medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (7), nor is it simply an improved method for accurate neonatal detection of PKU with a false-positive rate 10-fold lower than the best method previously available (8).
MADD patients showed the whole range of unusual fatty acids observed in both MCAD and vLCAD deficiencies, reflecting the generalized defect of all the mitochondrial acyl-CoA dehydrogenases.
ACADM, a member of the acyl-CoA dehydrogenase (ACAD) family that comprises 9 known proteins, is involved in the oxidation of medium-chain fatty acids and amino acids (Kim Miura, 2004).
Deficiencies have been identified in each of these FAO enzyme forms with the exception of long-chain acyl-CoA dehydrogenase (5).
However, the frequency of this mutation is considerably less than the A985 [right arrow] G mutation in medium-chain acyl-CoA dehydrogenase deficiency; thus, screening for the G1528 [right arrow] C mutation alone will result in considerable underdiagnosis of LCHAD deficiency.

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