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This Concept Map, created with IHMC CmapTools, has information related to: 11.21 - Biochem - PDH deficiency (Darcy), Ketone Bodies in Liver, Random X inactivation in different tissues, TCA cycle when lacking leads to fewer NADH molecules, oxidative decarboxylation of pyruvate to Acetyl CoA decreasing amount of substrate for TCA cycle, Pyruvate Dehydrogenase when inactivated reduces ATP/ADP ratio, primarily oxidative tissues specifically CNS, abnormal function which often results in neurological disorders, PDC deficiency prevents oxidation of glucose, E1 alpha-protein detected by Western Blot consistent with Missense Point mutation, posttranslational modifications by Pyruvate Dehydrogenase Kinase, decreased E1-alpha activity in Pyruvate Dehydrogenase, 10% E1 activity in fibroblasts illustrates decreased E1-alpha activity, B-Oxidation to form Ketone Bodies, Missense Point mutation leading to no reduction of E1-alpha protein or mRNA, decreased ratios of Acetyl CoA/CoA in mitochondria decreasing activity of Pyruvate Dehydrogenase Kinase, Pyruvate Dehydrogenase highly regulated by posttranslational modifications, oxidative decarboxylation of pyruvate to Acetyl CoA leading to elevated pyruvate in cytosol, disease management includes thiamine, x-chromosome representing X-Linked Mutation, Dichloroacetate counteracts Elevated Lactate levels