The following points highlight the seven major inborn errors of metabolism. The inborn errors are: 1. Phenylketonuria (PKU) 2. Galactosemia 3. Refsum’s Disease 4. Maple-Syrup Urine Disease 5. Fructose Intolerance 6. Von Gierke’s Disease 7. Hyperlipidemia. Metabolism: Inborn Error # 1. Phenylketonuria (PKU): PKU occurs due to a defect in the metabolism of amino acid […]
Major Inborn Errors of Metabolism
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Oxidation of Pyruvate to Acetyl-CoA
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In this article we will discuss about the Oxidation of Pyruvate to Acetyl-CoA. 1. Pyruvate oxidatively decarboxylated to acetyl-CoA (“active acetate”) before entering the citric acid cycle. 2. The reaction is catalysed by the multi-enzyme complex consisting of several different enzymes. This complex is known as pyruvate dehydrogenase complex. 3. Pyruvate is decarboxylated in the […]