Recent advances in the pathogenesis of hereditary fructose intolerance: implications for its treatment and the understanding of fructose-induced non-alcoholic fatty liver disease.

Recent advances in the pathogenesis of hereditary fructose intolerance: implications for its treatment and the understanding of fructose-induced non-alcoholic fatty liver disease. Cell Mol Life Sci. 2019 Nov 12;: Authors: Buziau AM, Schalkwijk CG, Stehouwer CDA, Tolan DR, Brouwers MCGJ 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 fru...
Source: Cellular and Molecular Life Sciences : CMLS - Category: Cytology Authors: Tags: Cell Mol Life Sci Source Type: research

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Authors: Arab JP, Dirchwolf M, Álvares-da-Silva MR, Barrera F, Benítez C, Castellanos-Fernandez M, Castro-Narro G, Chavez-Tapia N, Chiodi D, Cotrim H, Cusi K, de Oliveira CPMS, Díaz J, Fassio E, Gerona S, Girala M, Hernandez N, Marciano S, Masson W, Mendez-Sanchez N, Leite N, Lozano A, Padilla M, Panduro A, Paraná R, Parise E, Perez M, Poniachik J, Restrepo JC, Ruf A, Silva M, Tagle M, Tapias M, Torres K, Vilar-Gomez E, Costa Gil JE, Gadano A, Arrese M Abstract Non-alcoholic fatty liver disease (NAFLD) currently represents an epidemic worldwide. NAFLD is the most frequently diagnosed chr...
Source: Annals of Hepatology - Category: Gastroenterology Tags: Ann Hepatol Source Type: research
Hepatocellular carcinoma (HCC) has been increasing in incidence and prevalence for more than 30 years. As hepatologists have become more adept at treating underlying liver disease and cirrhosis, patients with cirrhosis are living longer, and with longer duration of cirrhosis comes an increased risk of HCC development. Cirrhosis is the underlying disease in most patients with HCC, although around 10% of HCC occurs without cirrhosis, often in the setting of chronic hepatitis B infection. Unfortunately, even with improving treatments for viral hepatitis, cirrhosis has continued to increase worldwide, due to the epidemic of no...
Source: Clinics in Liver Disease - Category: Gastroenterology Authors: Tags: Preface Source Type: research
In conclusion, long-term feeding of either HFHF or WDHC are reliable methods to induce NASH and diet-potentiated liver cancer in mice of both sexes; however, the choice of diet involves a trade-off between severity of metabolic syndrome and liver damage. PMID: 32924526 [PubMed - as supplied by publisher]
Source: Am J Physiol Endocri... - Category: Endocrinology Authors: Tags: Am J Physiol Endocrinol Metab Source Type: research
AbstractPurpose of ReviewAs the non-alcoholic fatty liver disease (NAFLD) epidemic continues to grow, more patients will be seeking advice on the safety of low to moderate alcohol use in the setting of underlying liver disease. Data suggests a beneficial effect in the general population, but whether this can be extended to the NAFLD population remains unclear. The purpose of this review is to summarize pertinent data in order to help guide medical decision-making and patient counseling.Recent FindingsNewer data suggests light to moderate alcohol use in patients with NAFLD can improve all-cause mortality, as well as cardiov...
Source: Current Hepatitis Reports - Category: Infectious Diseases Source Type: research
Authors: Kjær MB, George J, Kazankov K, Grønbæk H Abstract INTRODUCTION: Metabolic associated fatty liver disease (MAFLD) is a new nomenclature for fatty liver replacing non-alcoholic fatty liver disease (NAFLD). MAFLD has emerged as the leading cause of liver-related morbidity and mortality with increasing incidence due to its close association with the global epidemic of obesity and type 2 diabetes mellitus. Macrophages play a key role in MAFLD development and progression of steatohepatitis and fibrosis. Therefore, targeting macrophages may be a new therapeutic approach for MAFLD and MAFLD with...
Source: Expert Review of Gastroenterology and Hepatology - Category: Gastroenterology Tags: Expert Rev Gastroenterol Hepatol Source Type: research
With the obesity epidemic reaching crisis levels, there has been attention around those who may be resilient to the effects of obesity, termed metabolically healthy obesity (MHO), who initially present without...
Source: BMC Endocrine Disorders - Category: Endocrinology Authors: Tags: Research article Source Type: research
The rising tide of non-alcoholic fatty liver disease (NAFLD) associated with the obesity epidemic is a major international health concern. NAFLD is the leading global cause of liver disease with an estimated prevalence of 25% and is the fastest growing indication for liver transplantation (LT). The presence and severity of liver fibrosis is the only histologic predictor of clinical outcomes in this group. NAFLD poses several challenges in the peri-transplant setting including the management of multiple metabolic co-morbidities, post-transplant obesity and cardiovascular risk.
Source: Metabolism - Clinical and Experimental - Category: Biomedical Science Authors: Source Type: research
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Source: Gastrointestinal Endoscopy - Category: Gastroenterology Authors: Tags: Poster abstracts Source Type: research
co Giannini Hepatocellular carcinoma (HCC), the most frequent primary liver cancer, is the sixth most common cancer, the fourth leading cause of cancer-related deaths worldwide, and accounts globally for about 800,000 deaths/year. Early detection of HCC is of pivotal importance as it is associated with improved survival and the ability to apply curative treatments. Chronic liver diseases, and in particular cirrhosis, are the main risk factors for HCC, but the etiology of liver disease is rapidly changing due to improvements in the prevention and treatment of HBV (Hepatitis B virus) and HCV (Hepatitis C virus) infect...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Review Source Type: research
This study provides direct evidence for the contribution of gut microbiota to the cognitive decline during normal aging and suggests that restoring microbiota homeostasis in the elderly may improve cognitive function. On Nutraceutical Senolytics https://www.fightaging.org/archives/2020/05/on-nutraceutical-senolytics/ Nutraceuticals are compounds derived from foods, usually plants. In principle one can find useful therapies in the natural world, taking the approach of identifying interesting molecules and refining them to a greater potency than naturally occurs in order to produce a usefully large therape...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
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