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Fig. 1 | Orphanet Journal of Rare Diseases

Fig. 1

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

Fig. 1

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

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172