Mitochondrial betha-oxidation: genetic deficiency and therapy

The mitochondrion is the powerhouse of the cell, where energy is generated. Energy can be generated from the oxidation of proteins, polysaccharides and fats supplied by consuming food. Sugars, fatty acids and several amino acids are converted into the acetyl unit of acetyl-CoA is donationg its acetyl units to the citric acid cycle, whre oxidation to CO2 is taking place. Adenosine triphosphate (ATP) is generated as electrons flow to oxygen upon oxidative phosphorylation. Zie: Summary