In: Biology
Beta Oxidation:
Beta-oxidation is a crucial catabolic pathway which helps in breaking down the fatty acid molecules into acetyl-CoA. This step usually takes place in the cytosol in prokaryotes and the mitochondria in Eukaryotes. The generated acetyl- CoA enters the citric acid cycle producing NADH and FADH2 which will further be used in the electron transport chain. The name "Beta oxidation" is due to the oxidation of beta carbon of fatty acid to a carbonyl group.
If a person was unable to utilize beta-oxidation, because of specific mutations, the following complications may occur :
Mutations in the HADHA gene causes inadequate levels of 3-Hydroxyacyl-CoA dehydrogenase. If there are no sufficient levels of this enzyme[ referred to as LCHAD deficiency], long-chain fatty acids from the food and the body fat cannot be metabolized further. As a result of which the fatty acids cannot be converted into energy leading to several disorders such as hypoglycemia, hypotonia and lethargy.
The resultant long-chain fatty acids will deposit in the tissues and damage liver, causes muscle pain due to breakdown of muscle tissues and abnormalities in the retina and the nervous system. Additionally, it also causes heart and breathing problems and sometimes an unexpected sudden death may also occur. LCHAD deficiency can be triggered by fasting and due to viral infections.