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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 nattÅ, [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]
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 ...
The enzyme encoded by the MCAT gene, along with other enzymes that regulate Malonyl-CoA concentration, have been shown to regulate levels such that malonyl-CoA concentration decreases in human muscle tissue when under exercise training. This enzyme specifically has increased activity under these conditions, as it is known to catabolize malonyl-CoA.
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 .
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]
Mutations of the BCKDK gene, whose protein product controls the activity of the complex, may result in over-activation of the complex and excessive catabolism of the three amino acids. This leads to branched-chain keto acid dehydrogenase kinase deficiency, a rare disease first described in humans in 2012. [21]
General chemical structure of an acyl-CoA, where R is a carboxylic acid side chain. Acyl-CoA is a group of CoA-based coenzymes that metabolize carboxylic acids. Fatty acyl-CoA's are susceptible to beta oxidation, forming, ultimately, acetyl-CoA. The acetyl-CoA enters the citric acid cycle, eventually forming several equivalents of ATP. In this ...
Oxidation by FAD; Hydration; Oxidation by NAD + Thiolysis; Production of acyl-CoA and acetyl-CoA; The final product of β-oxidation of an even-numbered fatty acid is acetyl-CoA, the entry molecule for the citric acid cycle. [3] If the fatty acid is an odd-numbered chain, the final product of β-oxidation will be propionyl-CoA.