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.
Amino acid sequence similarities of the mitochondrial short chain [[DELTA].sup.3],[[DELTA].sup.2]-Enoyl-CoA isomerase and peroxisomal multifunctional [[DELTA].sup.3],[[DELTA].sup.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?
Deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase: a cause of lethal myophaty and cardiomyophaty in early childhood.
Long-chain 3-hydroxyacyl-CoA dehydrogenase: clinical presentation and follow up of 50 patients.
Five enzymes/proteins were identified as being differentially expressed in the livers of ketotic cows: expression of 3-hydroxyacyl-CoA dehydrogenase type-2 (HCDH), acetylcoenzyme A acetyltransferase 2 (ACAT) and elongation factor Tu (EF-Tu) were down-regulated, whereas that of alpha-enolase and creatine kinase were up-regulated.
Use of proper standards enabled us to identify this signal as 3-HG, an important metabolite accumulating in glutaric aciduria type I and short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (11).
It is important to note than increased C14 and C14:1 may be produced in other disorders as well, such as carnitine palmitoyltransferase II (CPT II), carnitine/acylcarnitine translocase (CACT), MADD, and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency.
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 >0.5 (14)].
Human liver long-chain 3-hydroxyacyl-CoA dehydrogenase is a multifunctional membrane-bound [beta]-oxidation enzyme of mitochondria.