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This Concept Map, created with IHMC CmapTools, has information related to: MCAD, carnitine deficiency leads to Inability to transport LCFAs into mitochondria, Acetyl CoA when decreased leads to increased Glycolysis, Omega-Oxidation is an alternative to beta oxidation, medium chain acyl CoA Dehydrogenase deficiency leads to build up NAD, Ketone Bodies made in the liver include acetoacetate, Ketone Bodies are transported from the liver to peripheral tissue where they are reconverted to acetyl CoA, gluconeogenesis when inhibited the patient uses existing glucose, Enoyl CoA is converted to 3-hydroxyacyl CoA, mitochondrial matrix where carnitine palmitoyl-transferase II catalyzes the synthesis, NAD build of up leads to high ratio of NAD/NADH, medium chain acyl CoA Dehydrogenase results in decreased breakdown of medium chain fatty acids, Hormone sensitive Lipase hydrolyses TAGs to Fatty Acids, TCA cycle to produce ATP, Glycolysis for production of ATP, fasting state ~12-24hrs low insulin, Inability to transport LCFAs into mitochondria results in HYPOKETOIC HYPOGLYCEMIA, gluconeogenesis is inhibited due to decreased Acetyl CoA, Unresponsive Child in a fasting state ~12-24hrs, medium chain acyl CoA Dehydrogenase deficiency leads to decreased Acetyl CoA, dicarboxylic acid specific example is succinic acid