B -Ketoacyl-CoA thiolase appeared as a single band of 45.5-kDa molecular mass on SDS/PAGE. The enzyme was purified 390-fold with 7% recovery. The native enzyme is a multimeric protein of a molecular mass of approximately of 182 kDa consisting of four identical subunits of 45.5 kDa, as ...
The crystal structure of the fatty acid elongating enzyme beta-ketoacyl [acyl carrier protein] synthase I (KAS I) from Escherichia coli has been determined to 2.3 A resolution by molecular replacement using the recently solved crystal structure of KAS II as a search model. The crystal contains ...
Deficient mitochondrial acetoacetyl-CoA thiolase in fibroblasts from four patients with 3-ketothiolase deficiency was studied using immunochemical methods. We also examined fibroblasts from two heterozygotes, the mother and the brother o... H Nagasawa,S Yamaguchi,T Orii,... - 《Pediatric Research》...
3.1Thiolases Threethiolases, 3-oxoacyl-CoAthiolase, β-ketoacyl-ACPsynthase(KAS) and 3-hydroxyl-3-methylglutaryl-CoA synthase (HMGS), are the most commonly usedenzymesin the Claisen-condensation based CoA-dependent carbon chain elongation process (Haapalainen et al., 2006;Harijan, 2017)....
Beta-Ketoacyl-ACP Synthase I beta-ketobutyric acid beta-ketobutyric acid beta-ketobutyric acid beta-ketobutyric acid beta-ketohydrogenase Beta-ketopropane Beta-ketopropane Beta-ketopropane beta-ketoreductase beta-ketothiolase Beta-ketothiolase deficiency beta-lactam beta-lactam beta-lactam Beta-lactam ...
Mitochondrial trifunctional protein (MTP/TFP) deficiency is thought to be a result of impaired fatty acid oxidation (FAO) due to mutations in hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase subunit A (HADHA HADHA/LCHAD) or subunit B (HADHB)1. A major phenotype of MTP...
Abbreviations: DAB, 3, 3′-diaminobenzidine tetrahydrochloride DHA, docosahexaenoic acid KT, 3-ketoacyl-CoA thiolase LC, long chain LCHAD, long-chain 3-hydroxyacyl-CoA dehydrogenase MEM, minimum essential medium MTP, mitochondrial trifunctional protein RPE, retinal pigment epithelium SC, short chain DOI...
The mitochondrial enoyl-CoA hydratase /3-hydroxyacyl-CoA dehydrogenase /3-ketoacyl-CoA thiolase trifunctional protein (trifunctional protein) plays a major... M Gharsalli,M Hajji - 《Comptes Rendus De Lacademie Des Sciences Serie Sciences De La Vie》 被引量: 5发表: 1992年 Abdominal fat depositi...
In this study, we have investigated whether the SCP2-related part of the 80 kDa protein leads to an intrinsic sterol and phospholipid transfer activity, as shown earlier for the 60 kDa SCP2-related peroxisomal 3-ketoacyl CoA thiolase with intrinsic sterol and phospholipid transfer activity called...