3-hydroxyacyl-CoA dehydrogenase

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3-hy·drox·y·ac·yl-CoA de·hy·dro·gen·ase

(hī-drok'sē-as'il dē'hī-drō'jen-ā),
β-Hydroxyacyl dehydrogenase; an enzyme catalyzing the oxidation of an l-3-hydroxyacyl-CoA to a 3-ketoacyl-CoA with the concomitant reduction of NAD+; one of the enzymes of the β oxidation of fatty acids.
References in periodicals archive ?
Acylcarnitines in fibroblasts of patients with lonhg-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and other fatty acid oxidation disorders.
The enzymes for the b-oxidation oflong- to mediumchain fatty acids (C-18 to C-12) are located close to the inner mitochondrial membrane, they are: very-longchain acyl-CoA dehydrogenase, long-chain enoyl-CoA hydratase, long-chain L 3-hydroxyacyl-CoA dehydrogenase and long-chain 3-ketoacyl-CoA thiolase.
2]-Enoyl-CoA isomerase, 2-Enoyl-CoA hydratase, 3-Hydroxyacyl-CoA dehydrogenase enzyme in rat liver.
Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: a novel glucose-fatty acid cycle?
Molecular characterization of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: formation of the enzyme complex is important for stabilization of both [alpha]--and [beta]-subunits.
The expression levels of 3-hydroxyacyl-CoA dehydrogenase type-2 (HCDH), acetyl-Coenzyme A acetyltransferase 2 (ACAT) and elongation factor Tu were down-regulated, and alpha-enolase and creatine kinase were up-regulated in the livers of ketotic cows.
The diagnoses made were long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (confirmed by mutation analysis), carnitine transporter defect (confirmed by cultured fibroblast fatty acid oxidation), and citrullinemia (confirmed by cultured fibroblast citrulline incorporation).
In this latter case, the pattern could mimic a long-chain 3-hydroxyacyl-CoA dehydrogenase or a trifunctional protein deficiency profile, except for the very high excretion of ketone bodies [in fatty acid oxidation defects, ketone bodies may appear increased in urine during fasting, but the ketosis remains at an inappropriately low level and the ratio of urinary adipate to 3-hydroxybutyrate is >0.
Human liver long-chain 3-hydroxyacyl-CoA dehydrogenase is a multifunctional membrane-bound [beta]-oxidation enzyme of mitochondria.
Seven plasma samples from five children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency were analyzed.
Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency in muscle: a new cause of recurrent myoglobinuria and encephalopathy.