Towards enhanced understanding of idiopathic ketotic hypoglycemia: a literature review and introduction of the patient organization Ketotic Hypoglycemia International

摘要

Selected details on the glucose and ketone body metabolism. Representation of selected details of the metabolism at the low insulin—high insulin counter-acting hormones state. Only cells of the liver, fat tissue and major fuel consuming organs are shown. Gluconeogenesis from amino acids, lactate/Cori cycle, and pathway details are omitted. Green line: Mitochondrial membrane. Blue circle: Citric acid cycle. Key enzymes in ketogenesis (arrows): (1) triglycerate lipase; (2) acetyl CoA carboxylase; (3) HMG CoA synthase. Key enzymes in ketolysis: (4) CoA-oxoacid transferase (SCOT); (5) michochondrial acetoacetyl CoA thiolase (MAT; beta-ketothiolase). Ac acetone, AcAc acetoacetate, ATP adenosine triphosphate, BOHB beta-hydroxybutyrate, FA fatty acids, OA oxaloacetate, TG triglyceride