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Metabolism and Genetic Disorders of Fructose: Pathophysiology, Diagnosis, and Treatment

This paper delves into the metabolism and genetic disorders related to fructose, exploring pathophysiology, diagnosis, and treatment options.

Ethan Brown
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Metabolism and Genetic Disorders of Fructose: Pathophysiology,Diagnosis, and TreatmentDescribe the metabolic pathways involved in fructose metabolism, highlighting the keyenzymes,theirroles,andtheconsequencesofinheritedenzymedeficiencies,suchasfructokinase and aldolase B deficiencies. Include information on the clinical manifestations,diagnosis, and treatment strategies for these conditions. Your answer should be approximately800-1000 words.

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Significant amounts of fructose, which is formed by cleaving a sucrose beforeentering the portal vein system, converted to glucose in the gut cells. Another partof the fructose is absorbed via transfer protein, i.e. lightweight by diffusion.Figure 1.Metabolism of fructoseMetabolism of fructose (Figure 1) starts with the phosphorylation reactioncatalyzed by fructokinase with the formation of fructose-1-phosphate. The enzymeis found in liver and kidney and intestine. This enzyme has an absolute specificity,however,unlikeglucokinase,insulindoesnotaffectitsactivity.Thelattercircumstance explains why the level of excretion of fructose in the urine of patientswith diabetes and healthy is no different. Fructose-1-phosphate can be converted tofructose-6-phosphatebecauseofthelackofappropriateenzyme.Insteadoffructose-1-phosphateiscleavedmorefructo-1-fosfataldolazoy(aldolaseB)dihydroxyacetone and glyceraldehyde-3-phosphate. The latter is the intermediateofglycolysisandisformedinthereactioncatalyzedbyfructose-1,6-bisfosfosfataldolazoy (aldolase A). Glyceraldehyde may be included in glycolysisafter phosphorylation with ATP. Or two triose phosphate molecules decompose onthe glycolytic pathway, or condensed to form fructose 1,6-bisphosphate andmore
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