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The first oxidation step in the peroxisome is catalyzed by the enzyme acyl-CoA oxidase. The β-ketothiolase used in peroxisomal β-oxidation has an altered substrate specificity, different from the mitochondrial β-ketothiolase. Peroxisomal oxidation is induced by a high-fat diet and administration of hypolipidemic drugs like clofibrate.
The carnitine palmitoyltransferase system is an essential step in the beta-oxidation of long chain fatty acids. This transfer system is necessary because, while fatty acids are activated (in the form of a thioester linkage to coenzyme A) on the outer mitochondrial membrane, the activated fatty acids must be oxidized within the mitochondrial matrix
The resulting acyl-CoA cross the mitochondria membrane and enter the process of beta oxidation. The main products of the beta oxidation pathway are acetyl-CoA (which is used in the citric acid cycle to produce energy), NADH and FADH. [16] The process of beta oxidation requires the following enzymes: acyl-CoA dehydrogenase, enoyl-CoA hydratase ...
The regulation of mammalian ACC is complex, in order to control two distinct pools of malonyl-CoA that direct either the inhibition of beta oxidation or the activation of lipid biosynthesis. [14] Mammalian ACC1 and ACC2 are regulated transcriptionally by multiple promoters which mediate ACC abundance in response to the cells nutritional status.
Malonyl-CoA is a highly regulated molecule in fatty acid synthesis; as such, it inhibits the rate-limiting step in beta-oxidation of fatty acids. Malonyl-CoA inhibits fatty acids from associating with carnitine by regulating the enzyme carnitine acyltransferase, thereby preventing them from entering the mitochondria, where fatty acid oxidation and degradation occur.
Five of these nine classes are involved in fatty acid β-oxidation (SCAD, MCAD, LCAD, VLCAD, and VLCAD2), and the other four are involved in branched chain amino acid metabolism (i3VD, i2VD, GD, and iBD). Most acyl-CoA dehydrogenases are α 4 homotetramers, and in two cases (for very long chain fatty acid substrates) they are α 2 homodimers ...
Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, LCHAD deficiency. Mitochondrial trifunctional protein (MTP) is a protein attached to the inner mitochondrial membrane which catalyzes three out of the four steps in beta oxidation.
15107 Ensembl ENSG00000138796 ENSMUSG00000027984 UniProt Q16836 Q61425 RefSeq (mRNA) NM_001184705 NM_005327 NM_001331027 NM_008212 RefSeq (protein) NP_001171634 NP_001317956 NP_005318 NP_032238 Location (UCSC) Chr 4: 107.99 – 108.04 Mb Chr 3: 131.03 – 131.07 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Hydroxyacyl-Coenzyme A dehydrogenase (HADH) is an enzyme which in humans is ...