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References in periodicals archive ?
Dietary Management of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD).
19) In addition to its effect on palmitoyl-CoA and acyl coenzyme A oxidase, sesamin was also noted to amplify the action of numerous other hepatic fatty acid oxidation enzymes, including carnitine palmitoyltransferase, acyl-CoA dehydrogenase, 3-hydroxyacyl-CoA dehydrogenase, enoyi-CoA hydratase and 3-ketoaeyl-CoA thiolase, as well as to improve, in a dose-dependent manner, the activity of 2,4-dienoyl-CoA reductase and [DELTA]3, [DELTA]2-enoyl-CoA isomerase, both of which are involved in the auxiliary pathway for beta-oxidation of unsaturated fatty acids.
The second step is carried out by an enoyl-CoA hydratase, and the enzyme involved in the third step is an L 3-hydroxyacyl-CoA dehydrogenase using NAD as cofactor for the reaction, which is reduced to NADH transferring electrons to complex 1 of the respiratory chain.
Peroxisomal bifunctional protein from rat liver is a trifunctional enzyme possessing 2-Enoyl-CoA hydratase, 3-Hydroxyacyl-CoA dehydrogenase, and [[DELTA].
Likewise, enzyme activities of 3-hydroxyacyl-CoA dehydrogenase and carnityl palmitoyl-CoA transferase are good indicators of lipid metabolism.
2; encoded by KCNJ11) (7); glucokinase (GK; encoded by GCK) (8); glutamate dehydrogenase (GDH; encoded by GLUD-1) (9); short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD; encoded by HADH) (10); and ectopic expression on [beta]-cell plasma membrane of SLC16A1 [encodes monocarboxylate transporter 1 (MCT1)] (11).
The disease has been associated with a deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD).
In our previous research using comparative proteomic techniques, we observed that the expression of acetyl-Coenzyme A acetyltransferase 2 and 3-hydroxyacyl-CoA dehydrogenase type-2, those enzymes involved in fatty acid degradation, were decreased in the liver tissues of ketotic cows, which suggested that the ability to utilize fatty acids to produce C[O.
The 3 enzymes involved are long-chain enoyl-CoA hydratase (LCEH), long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), and long-chain 3-ketoacyl-CoA thiolase (LCTH).
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.