Inborn Errors of Fructose Metabolism. What Can We Learn from Them?
AbstractFructose 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. View Full-Text
Scifeed alert for new publicationsNever miss any articles matching your research from any publisher
- Get alerts for new papers matching your research
- Find out the new papers from selected authors
- Updated daily for 49'000+ journals and 6000+ publishers
- Define your Scifeed now
Tran, C. Inborn Errors of Fructose Metabolism. What Can We Learn from Them? Nutrients 2017, 9, 356.
Tran C. Inborn Errors of Fructose Metabolism. What Can We Learn from Them? Nutrients. 2017; 9(4):356.Chicago/Turabian Style
Tran, Christel. 2017. "Inborn Errors of Fructose Metabolism. What Can We Learn from Them?" Nutrients 9, no. 4: 356.
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.