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Acetoacetate (AcAc) and 3-hydroxybutyrate (3HB), the two main ketone bodies of humans, are important vectors of energy transport from the liver to extrahepatic tissues, especially during fasting, when glucose supply is low. Blood total ketone body (TKB) levels should be evaluated in the context of clinical history, such as fasting time and ketogenic stresses. Blood TKB should also be evaluated in parallel with blood glucose and free fatty acids (FFA). The FFA/TKB ratio is especially useful for evaluation of ketone body metabolism. Defects in ketogenesis include mitochondrial HMG-CoA synthase (mHS) deficiency and HMG-CoA lyase (HL) deficiency. mHS deficiency should be considered in non-ketotic hypoglycemia if a fatty acid beta-oxidation defect is suspected, but cannot be confirmed. Patients with HL deficiency can develop hypoglycemic crises and neurological symptoms even in adolescents and adults. Succinyl-CoA-3-oxoacid CoA transferase (SCOT) deficiency and beta-ketothiolase (T2) deficiency are two defects in ketolysis. Permanent ketosis is pathognomonic for SCOT deficiency. However, patients with "mild" SCOT mutations may have nonketotic periods. T2-deficient patients with "mild" mutations may have normal blood acylcarnitine profiles even in ketoacidotic crises. T2 deficient patients cannot be detected in a reliable manner by newborn screening using acylcarnitines. We review recent data on clinical presentation, metabolite profiles and the course of these diseases in adults, including in pregnancy.
Authors : Fukao Toshiyuki , Mitchell Grant , Sass Jörn Oliver , Hori Tomohiro , Orii Kenji , Aoyama Yuka ,
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WP1817: Fatty acid, triacylglycerol, and ketone body metabolism
WP2316: PPAR signaling pathway
WP100: Glutathione metabolism
WP101: Synthesis and Degradation of Ketone Bodies
WP1015: Synthesis and Degradation of Ketone Bodies
WP1026: One Carbon Metabolism
WP1030: Selenium metabolism Selenoproteins
WP1038: Vitamin A and carotenoid metabolism
WP1039: Glutathione metabolism
WP104: Alanine and aspartate metabolism
WP1053: Estrogen metabolism
WP106: Alanine and aspartate metabolism
WP1073: Glycogen Metabolism
WP108: Selenium metabolism/Selenoproteins
WP1099: Nuclear receptors in lipid metabolism and toxicity
WP112: Principle Pathways of Carbon Metabolism
WP1126: Tryptophan metabolism
WP1134: Synthesis and Degradation of Ketone Bodies
WP1145: One Carbon Metabolism
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WP1155: Vitamin A and carotenoid metabolism
WP1169: Estrogen metabolism
WP1189: Glycogen Metabolism
WP1191: Nucleotide Metabolism
WP1203: One Carbon Metabolism
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