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This Concept Map, created with IHMC CmapTools, has information related to: HMG CoA Lyase, Decreased ATP Synthesis causes Decreased Glycogen Production, 3-HMG-CoA leading to Decreased Acetoacetate, Decreased Beta-Oxidation (in the liver) leads to Excess Lipid in Hepatocytes, Decreased Glycogen Production caused by inhibition of Glycogenesis (regulated by insulin), HMG-CoA Lyase Deficiency is a deficiency of HMG-CoA Lyase, UTP leading to decreased Glycogen Stores (small in infants), Gluconeogenesis (regulated by Glucagon) rate limited by Carbamoyl Phosphate Synthetase I, 3-HMG-CoA leading to Decreased Acetyl-CoA, L-Carnitine transports Amino Acids Across Mitochondrial Membrane, Glycogen Stores (small in infants) exhausted by Glycognolysis (regulated by glucagon), glycogen via Glycogen Phosphorylase, Gluconeogenesis (regulated by Glucagon) requires Carbon Skeletons, Branched Chain Ketogenic Amino Acid not used in Gluconeogenesis (regulated by Glucagon), Decreased ATP Synthesis causes Decreased Urea Synthesis, Gluconeogenesis (regulated by Glucagon) requires ATP, Excess Substrates including Acetoacetyl, Decreased Acetoacetate leading to Decreased B-Hydroxybutyrate, Patient has HMG-CoA Lyase Deficiency, 3-Methylcrotonyl-CoA seen in Abnormal Urine Sample, L-Carnitine often decreased due to Limited Protein Diet