ReviewCellular and molecular life sciences : CMLS2020
Recent advances in the pathogenesis of hereditary fructose intolerance: implications for its treatment and the understanding of fructose-induced non-alcoholic fatty liver disease.
Review in Cellular and molecular life sciences : CMLS, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 54 citations in OpenAlex.
- Delayed diagnosis of hereditary fructose intolerance presenting as chronic lean steatosis in an adolescent.JPGN reports · 2026Article
- When Fruit Turns Harmful: Late Diagnosis of Hereditary Fructose Intolerance in a Pediatric Patient-A Case Report and Literature Review.Case reports in medicine · 2026Article
- Proteomics Used in Identifying Novel Correlates of Disease in Pediatric Metabolic Dysfunction-Associated Steatotic Liver Disease.Clinical and translational science · 2025Article
- Safety and efficacy of pharmacological inhibition of ketohexokinase in hereditary fructose intolerance.The Journal of clinical investigation · 2025Article
- Aldolase B Deficient Mice Are Characterized by Hepatic Nucleotide Sugar Abnormalities.Journal of inherited metabolic disease · 2025Article
- Hepatic glucokinase regulatory protein and carbohydrate response element binding protein attenuation reduce de novo lipogenesis but do not mitigate intrahepatic triglyceride accumulation in Aldob deficiency.Molecular metabolism · 2024Article
- A Case Study of a Rare Disease (Fructosemia) Diagnosed in a Patient with Abdominal Pain.Journal of clinical medicine · 2024Article
- Descriptive Analysis of Carrier and Affected Hereditary Fructose Intolerance in Women during Pregnancy.Healthcare (Basel, Switzerland) · 2024Article
- Clinical Practice Guidelines for the Diagnosis and Management of Hereditary Fructose Intolerance.Diseases (Basel, Switzerland) · 2024Article
- High dietary Fructose Drives Metabolic Dysfunction-Associated Steatotic Liver Disease via Activating ubiquitin-specific peptidase 2/11β-hydroxysteroid dehydrogenase type 1 Pathway in Mice.International journal of biological sciences · 2024Article
- Combined PMM2-CDG and hereditary fructose intolerance in a patient with mild clinical presentation.Molecular genetics and metabolism · 2023Article
- Estimation of hereditary fructose intolerance prevalence in the Chinese population.Orphanet journal of rare diseases · 2022Review
- Seasonal and sex-dependent gene expression in emu (Dromaius novaehollandiae) fat tissues.Scientific reports · 2022Article
- New Insights into the Chemical Composition of Ayahuasca.ACS omega · 2022Article
- Compendium of causative genes and their encoded proteins for common monogenic disorders.Protein science : a publication of the Protein Society · 2022Article
- Celiac Disease in Conjunction with Hereditary Fructose Intolerance as a Rare Cause of Liver Steatosis with Mild Hypertransaminasemia-A Case Report.Pediatric reports · 2021Article
- Correlation of Hepatic Steatosis Among Cohabitants Using Hounsfield Unit From Coronary Computed Tomography.Cureus · 2021Article
- Jejunal mucosa proteomics unravel metabolic adaptive processes to mild chronic heat stress in dairy cows.Scientific reports · 2021Article
- Targeting hepatocyte carbohydrate transport to mimic fasting and calorie restriction.The FEBS journal · 2021Review
- Fructose and metabolic diseases: too much to be good.Chinese medical journal · 2021Article
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Authors and funding
5 authors at 3 institutions in 2 countries.
Funding
Abstract
Hereditary fructose intolerance (HFI) is a rare inborn disease characterized by a deficiency in aldolase B, which catalyzes the cleavage of fructose 1,6-bisphosphate and fructose 1-phosphate (Fru 1P) to triose molecules. In patients with HFI, ingestion of fructose results in accumulation of Fru 1P and depletion of ATP, which are believed to cause symptoms, such as nausea, vomiting, hypoglycemia, and liver and kidney failure. These sequelae can be prevented by a fructose-restricted diet. Recent studies in aldolase B-deficient mice and HFI patients have provided more insight into the pathogenesis of HFI, in particular the liver phenotype. Both aldolase B-deficient mice (fed a very low fructose diet) and HFI patients (treated with a fructose-restricted diet) displayed greater intrahepatic fat content when compared to controls. The liver phenotype in aldolase B-deficient mice was prevented by reduction in intrahepatic Fru 1P concentrations by crossing these mice with mice deficient for ketohexokinase, the enzyme that catalyzes the synthesis of Fru 1P. These new findings not only provide a potential novel treatment for HFI, but lend insight into the pathogenesis of fructose-induced non-alcoholic fatty liver disease (NAFLD), which has raised to epidemic proportions in Western society. This narrative review summarizes the most recent advances in the pathogenesis of HFI and discusses the implications for the understanding and treatment of fructose-induced NAFLD.
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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 Socratic graph.