Ads
related to: branched chain fatty acid oxidation
Search results
Results From The WOW.Com Content Network
Branched-chain fatty acids (BCFA) are usually saturated fatty acids with one or more methyl branches on the carbon chain. [1] [2] BCFAs are most often found in bacteria, [2] but can be found in nattō, [3] dairy, [4] vernix caseosa of human infants and California sea lions [5] where they may play a role in fostering the development of their intestinal microbiota. [1]
The same enzymes are used in peroxisomes as in the mitochondrial matrix and acetyl-CoA is generated. Very long chain (greater than C-22) fatty acids, branched fatty acids, [9] some prostaglandins and leukotrienes [10] undergo initial oxidation in peroxisomes until octanoyl-CoA is formed, at which point it undergoes mitochondrial oxidation. [11]
Alpha oxidation (α-oxidation) is a process by which certain branched-chain [1] fatty acids are broken down by removal of a single carbon from the carboxyl end. In humans, alpha-oxidation is used in peroxisomes to break down dietary phytanic acid , which cannot undergo beta-oxidation due to its β-methyl branch, into pristanic acid .
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 ...
Short-chain fatty acids (SCFAs) are fatty acids with aliphatic tails of five or fewer carbons (e.g. butyric acid). [7] Medium-chain fatty acids (MCFAs) are fatty acids with aliphatic tails of 6 to 12 [8] carbons, which can form medium-chain triglycerides. Long-chain fatty acids (LCFAs) are fatty acids with aliphatic tails of 13 to 21 carbons. [9]
The BCKA decarboxylase enzyme is composed of two subunits in a tetrameric structure (A 2 B 2) and is essential for the synthesis of branched-chain fatty acids. It is responsible for the decarboxylation of α-keto acids formed by the transamination of valine, leucine, and isoleucine and produces the primers used for branched-chain fatty acid ...
Refsum disease is a peroxisomal disorder caused by the impaired alpha-oxidation of branched chain fatty acids resulting in buildup of phytanic acid and its derivatives in the plasma and tissues. This may be due to deficiencies of phytanoyl-CoA hydroxylase or peroxin-7 activity, encoded by the genes PHYH [ 10 ] and PEX7 , [ 11 ] respectively.
When a fatty acid oxidation disorder affects the muscles, it is a metabolic myopathy. Moreover, cancer cells can display irregular fatty acid metabolism with regard to both fatty acid synthesis [44] and mitochondrial fatty acid oxidation (FAO) [45] that are involved in diverse aspects of tumorigenesis and cell growth.