文章基本信息

标题：Inborn Errors of Fructose Metabolism. What Can We Learn from Them?
本地全文：下载
作者：Christel Tran
期刊名称：Nutrients
电子版ISSN：2072-6643
出版年度：2017
卷号：9
期号：4
页码：356
DOI：10.3390/nu9040356
语种：English
出版社：MDPI Publishing
摘要：Fructose is one of the main sweetening agents in the human diet and its ingestion is increasing globally. Dietary sugar has particular effects on those whose capacity to metabolize fructose is limited. If intolerance to carbohydrates is a frequent finding in children, inborn errors of carbohydrate metabolism are rare conditions. Three inborn errors are known in the pathway of fructose metabolism; (1) essential or benign fructosuria due to fructokinase deficiency; (2) hereditary fructose intolerance; and (3) fructose-1,6-bisphosphatase deficiency. In this review the focus is set on the description of the clinical symptoms and biochemical anomalies in the three inborn errors of metabolism. The potential toxic effects of fructose in healthy humans also are discussed. Studies conducted in patients with inborn errors of fructose metabolism helped to understand fructose metabolism and its potential toxicity in healthy human. Influence of fructose on the glycolytic pathway and on purine catabolism is the cause of hypoglycemia, lactic acidosis and hyperuricemia. The discovery that fructose-mediated generation of uric acid may have a causal role in diabetes and obesity provided new understandings into pathogenesis for these frequent diseases.
关键词：fructose; inborn errors of metabolism; toxicity fructose ; inborn errors of metabolism ; toxicity