What Are Common Fatty Acid Oxidation Metabolic Disorders?

Discussion All cells and particularly their mitochondria need an energy source. Glucose is one of the most common ones, but also fatty acids, lactate, pyruvate, ketones, and amino acids. Fatty acids are formed with a carboxylic acid with a long aliphatic carbon chain usually with even numbers of carbon atoms (usually 4-28 most commonly). Most are unbranched and in foods are usually found in the form of esters. Fatty acids are important energy sources for the heart (50-70%) but also skeletal muscle where resting muscle uses both glucose and fatty acids. During fasting or increased stress fatty acids become a major source of energy in skeletal muscle. Regulation is by several factors including physiological state, organ system, substrate and co-substrate availability (such as oxygen or carnitine), blood supply, hormones, etc. Long-chain fatty acids need the carnitine transfer system to transport the substance across the outer mitochondrial membrane. Medium and short-chain fatty acids enter the mitochondria directly. Therefore carnitine availability and metabolism is vital for long-chain fatty acids metabolism but not for shorter ones. Health problems occur when there is insufficient energy production and also build up of precursor metabolites. With the increase and expansion of neonatal screening programs, especially the use of tandem mass spectrometry, in the US, most infants are screened at birth for fatty acid metabolism problems and therefore can be treated very early in...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news

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