ReviewNutrients2017
Inborn Errors of Fructose Metabolism. What Can We Learn from Them?
Review in Nutrients, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 69 citations in OpenAlex.
- Intestinal fructose catabolism promotes obesity and insulin resistance via ileal lacteal remodeling.Science advances · 2026Article
- Review: Fructose, the Sweet Culprit behind Nonalcoholic Fatty Liver Disease and Type 2 Diabetes.Current diabetes reviews · 2026Review
- Progress in Understanding the Regulation of Glucose and Fructose Metabolism.Annual review of nutrition · 2025Review
- Intrafamilial phenotypic variability due to a missense pathogenic variant in FBP1 gene.Molecular genetics and metabolism reports · 2024Article
- Metabolic management of a successful pregnancy and postpartum complications in fructose-1,6-bisphosphatase deficiency.JIMD reports · 2024Article
- Sugar alcohol degradation in Archaea: uptake and degradation of mannitol and sorbitol in Haloarcula hispanica.Extremophiles : life under extreme conditions · 2024Article
- An extremely rare case of hypoglycemia with a novel mutation and review of the literature: fructose-1,6 bisphosphatase deficiency in an adult man.Irish journal of medical science · 2024Review
- Fructose-1,6-bisphosphatase deficiency: estimation of prevalence in the Chinese population and analysis of genotype-phenotype association.Frontiers in genetics · 2024Article
- Review
- Immunodeficiency with susceptibility to lymphoma with complex genotype affecting energy metabolism (Frontiers in immunology · 2023Article
- Estimation of hereditary fructose intolerance prevalence in the Chinese population.Orphanet journal of rare diseases · 2022Review
- Hereditary fructose intolerance: A comprehensive review.World journal of clinical pediatrics · 2022Review
- A novel variant of fructose-1,6-bisphosphatase gene identified in an adult with newly diagnosed hepatitis C.JIMD reports · 2022Article
- Tolerable upper intake level for dietary sugars.EFSA journal. European Food Safety Authority · 2022Article
- Fructose and Mannose in Inborn Errors of Metabolism and Cancer.Metabolites · 2021Review
- Neonatal Metabolic Acidosis in the Neonatal Intensive Care Unit: What Are the Genetic Causes?Frontiers in pediatrics · 2021Article
- Fructose Metabolism in Cancer.Cells · 2020Review
- Essential tremor: Is the word "essential" really essential?Parkinsonism & related disorders · 2020Article
- Tissue-Specific Fructose Metabolism in Obesity and Diabetes.Current diabetes reports · 2020Review
- Adaptation of the Gut Microbiota to Modern Dietary Sugars and Sweeteners.Advances in nutrition (Bethesda, Md.) · 2020Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
1 author.
Funding
No grant is acknowledged in the PubMed record.
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.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.