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.

Textbook of Biochemistry with Clinical Correlations, Sixth Edition

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.

Efecto del arilo del fruto de Blighia sapida sobre el glucogeno total del molusco Geukensia demissa

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.

Isolated mitochondrial long-chain ketoacyl-CoA thiolase deficiency resulting from mutations in the HADHB gene

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).

2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation

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).

Use of tandem mass spectrometry for multianalyte screening of dried blood specimens from newborns

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.

Simultaneous analysis of plasma free fatty acids and their 3-hydroxy analogs in fatty acid [beta]-oxidation disorders

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).

Differential expression of PPAR[gamma], FASN, and ACADM genes in various adipose tissues and longissimus dorsi muscle from Yanbian yellow cattle and Yan yellow cattle

Deficiencies have been identified in each of these FAO enzyme forms with the exception of long-chain acyl-CoA dehydrogenase (5).

Accumulation of free 3-hydroxy fatty acids in the culture media of fibroblasts from patients deficient in long-chain L-3-hydroxyacyl-CoA dehydrogenase: a useful diagnostic aid

Molecular characterization of inherited medium-chain acyl-CoA dehydrogenase deficiency.

Improved stable isotope dilution-gas chromatography-mass spectrometry method for serum or plasma free 3-hydroxy-fatty acids and its utility for the study of disorders of mitochondrial fatty acid [beta]-oxidation

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.

Stability of long-chain and short-chain 3-hydroxyacyl-CoA dehyd rogenase activity in postmortem liver

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