Next Article in Journal
High Fat Diets Sex-Specifically Affect the Renal Transcriptome and Program Obesity, Kidney Injury, and Hypertension in the Offspring
Next Article in Special Issue
Associations of Dietary Glucose, Fructose, and Sucrose with β-Cell Function, Insulin Sensitivity, and Type 2 Diabetes in the Maastricht Study
Previous Article in Journal
Analysis of the Anti-Cancer Effects of Cincau Extract (Premna oblongifolia Merr) and Other Types of Non-Digestible Fibre Using Faecal Fermentation Supernatants and Caco-2 Cells as a Model of the Human Colon
Previous Article in Special Issue
Role of the Enterocyte in Fructose-Induced Hypertriglyceridaemia
Open AccessReview

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

Center for Molecular Diseases, Division of Genetic Medicine, Lausanne University Hospital (CHUV), Beaumont-02/248, Lausanne CH-1011, Switzerland
Nutrients 2017, 9(4), 356;
Received: 24 February 2017 / Revised: 27 March 2017 / Accepted: 28 March 2017 / Published: 3 April 2017
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. View Full-Text
Keywords: fructose; inborn errors of metabolism; toxicity fructose; inborn errors of metabolism; toxicity
Show Figures

Figure 1

MDPI and ACS Style

Tran, C. Inborn Errors of Fructose Metabolism. What Can We Learn from Them? Nutrients 2017, 9, 356.

Show more citation formats Show less citations formats
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

Back to TopTop