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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.
In biochemistry, fatty acid synthesis is the creation of fatty acids from acetyl-CoA and NADPH through the action of enzymes called fatty acid synthases. This process takes place in the cytoplasm of the cell. Most of the acetyl-CoA which is converted into fatty acids is derived from carbohydrates via the glycolytic pathway.
Fatty acid synthesis starts with acetyl-CoA and builds up by the addition of two-carbon units. Fatty acid synthesis occurs in the cytoplasm of cells while oxidative degradation occurs in the mitochondria. Many of the enzymes for the fatty acid synthesis are organized into a multienzyme complex called fatty acid synthase. [5]
Accordingly, cancer cells can display irregular lipid metabolism with regard to both fatty acid synthesis and mitochondrial fatty acid oxidation (FAO) that are involved in diverse aspects of tumorigenesis and cell growth. [17] Several specific β-oxidation disorders have been identified.
Glucagon: stimulates fatty acid oxidation and inhibits de novo fatty acid synthesis, reducing VLDL release and hepatic steatosis. [ 21 ] Thyroid Hormone: promotes hepatic triglyceride synthesis, enhancing lipolysis, stimulating mitochondrial fatty acid β-oxidation, and regulating cholesterol levels through various mechanisms, including LDL ...
Example of Beta Oxidation using Stearic Acid. Beta oxidation, as well as alpha-oxidation, also occurs in the peroxisome. [1] The peroxisome handles beta oxidation of fatty acids that have more than 20 carbons in their chain because the peroxisome contains very-long-chain Acyl-CoA synthetases. [9] These enzymes are better equipped to oxidize ...
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.
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.