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31P-MRS of liver metabolite concentrations (mM) before (−) and after an intravenous bolus of fructose (200 mg/kg, in 2 min), in three healthy controls and in an adult proband with essential fructosuria (RK), hereditary fructose intolerance (VL), or fructose-1,6-bisphosphatase deficiency (TM). PME = phosphomonoesters; P = inorganic phosphate; ATP = adenosine triphosphate. Note that three fourths of the inorganic phosphorus present in liver remains invisible by MRS.218, 498 (Reprinted with permission from Boesiger P, Buchli R, Meier D, Steinmann B, Gitzelmann R. Pediatr Res 36:436, ) Source: Disorders of Fructose Metabolism, The Online Metabolic and Molecular Bases of Inherited Disease Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular Bases of Inherited Disease; 2014 Available at: Accessed: December 18, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved
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