Inborn Errors of Fructose Metabolism. What Can We Learn from Them?

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Ressource 1Download: nutrients-09-00356-v3.pdf (365.99 [Ko])
State: Public
Version: Final published version
Serval ID
serval:BIB_74F1B1039AC3
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Inborn Errors of Fructose Metabolism. What Can We Learn from Them?
Journal
Nutrients
Author(s)
Tran C.
ISSN
2072-6643 (Electronic)
ISSN-L
2072-6643
Publication state
Published
Issued date
03/04/2017
Peer-reviewed
Oui
Volume
9
Number
4
Pages
0
Language
english
Notes
Publication types: Journal Article ; Review
Publication Status: epublish
Abstract
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.

Pubmed
Web of science
Open Access
Yes
Create date
11/04/2017 17:46
Last modification date
20/08/2019 14:32
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