Role of ERAB/l-3-Hydroxyacyl-coenzyme A Dehydrogenase Type II Activity in Aβ-induced Cytotoxicity

[Long-chain 3-hydroxyacyl-coenzyme a dehydrogenase deficiency and cardiomyopathy].

Increased nuchal translucency in a case of long-chain 3-hydroxyacyl- coenzyme A dehydrogenase deficiency.

We present a case where the embryo showed an increased nuchal edema and a metabolic disorder. At 31 weeks of gestation the fetus developed a cardiomegaly and a hydrops. In this case, a long-chain 3-hydroxacyl-coenzyme A dehydrogenase deficiency (LCHAD deficiency) was confirmed by biochemical investigations in cultured chorionic villus cells and by DNA analysis. This metabolic disease causes a r...

Differences in the binding of coenzyme to L-3-hydroxyacyl-coenzyme A dehydrogenase in the crystalline state and in solution.

L-3-Hydroxyacyl coenzyme A dehydrogenase (L-3hydroxyacyl-CoA:NAD oxidoreductase; EC 1 .l .1.35) is a mitochondrial enzyme involved in the beta oxidation of fatty acids. The enzyme, as isolated from pig heart muscle, hasM, 67 000 and contains two identical subunits [l]. The amino acid sequence has recently been completed and appears to have little homology with other dehydrogenases [2]. Like man...

Refined staging for chorioretinopathy in long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency.

OBJECTIVE Neonatal screening and earlier diagnosis have improved the prognosis of long-chain 3-hydroxyacyl coenzyme A dehydrogenase (LCHAD) deficiency, which causes a need to refine the staging of the pigmentary chorioretinopathy and thus improve monitoring and comparability of patients under dietary therapy. METHODS Seven children with LCHAD deficiency caused by homozygous G1528C mutation at...

Re: Hadh2 and 3-hydroxyacyl-CoA dehydrogenase.

TO THE EDITOR: To investigate the impact of maternal diabetes on oocyte metabolism and meiotic maturation, Ratchford et al. (10) reported changes in 3-hydroxyacyl-CoA dehydrogenase II (Hadh2) activity in oocytes after treatment with 5-aminoimidazole-4-carboxamide-1-D-ribofuranoside or after administration of human chorionic gonadotropin, respectively (as shown in Figs. 3A and 4A of Ref. 10). Th...