Non-alcoholic fatty liver in hereditary fructose intolerance.

BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is characterized by fat accumulation affecting >5% of the liver volume that is not explained by alcohol abuse. It is known that fructose gives rise to NAFLD and it has been recently described that the ingestion of fructose in low amounts in aldolase B deficient mice is associated with the development of fatty liver. Therefore, it is reasonable that patients with HFI (Hereditary Fructose Intolerance) present fatty liver at diagnosis, but its prevalence in patients treated and with adequate follow-up is not well documented in the literature. The aim of this study is to analyze the association between HFI and NAFLD in treated patients. METHODS: A cross-sectional observational study was conducted. The population comprised 16 genetically diagnosed HFI patients aged from 3 years to 48 and in dietary treatment of fructose, sorbitol and sacarose exclusion at least for two years. Blood samples were obtained for analytical studies and anthropometric measurements of each patient were performed. RESULTS: Patients presented a Body Mass Index (BMI) of 17.9 ± 2.9 kg/m2. The HOMA index and Quick index were in normal range for our population. The S-adenosyl-methionine (SAM)/S-adenosyl-l-homocysteine (SAH) ratio was increased in the patients in whom this analysis was performed. By imaging techniques it was observed that 9 of the 16 patients presented fatty liver (7 by hepatic MRI). Of these 9 patients, only 3 presented hepatomegaly. 7 of 9 patients affected by the c.448G > C mutation had fatty infiltration, of which three of them presented in addition hepatomegaly. CONCLUSIONS: There is a high prevalence of fatty liver in HFI patients and it is not related to obesity and insulin resistance. The diagnosis of fatty liver in HFI patients and, above all, the identification of new therapeutic approaches, can positively impact the quality of life of these patients.

A sarabande of tropical fruit proteomics: Avocado, banana, and mango.

The present review highlights the progress made in plant proteomics via the introduction of combinatorial peptide ligand libraries (CPLL) for detecting low-abundance species. Thanks to a novel approach to the CPLL methodology, namely, that of performing the capture both under native and denaturing conditions, identifying plant species in the order of thousands, rather than hundreds, is now possible. We report here data on a trio of tropical fruits, namely, banana, avocado, and mango. The first two are classified as "recalcitrant" tissues since minute amounts of proteins (in the order of 1%) are embedded on a very large matrix of plant-specific material (e.g., polysaccharides and other plant polymers). Yet, even under these adverse conditions we could report, in a single sweep, from 1000 to 3000 unique gene products. In the case of mango the investigation has been extended to the peel too, since this skin is popularly used to flavor dishes in Far East cuisine. Even in this tough peel 330 proteins could be identified, whereas in soft peels, such as in lemons, one thousand unique species could be detected.

Simple and rapid characterization of soybean cultivars by perfusion reversed-phase HPLC: application to the estimation of the 11S and 7S globulin contents.

A perfusion RP HPLC method enabling the separation of soybean proteins in an analysis time lower than 3 min has been used to obtain the chromatographic profiles of different soybean cultivars. The chromatograms obtained for each soybean variety presented clear differences that justified the potential use of this method for cultivar characterization. The area percentages obtained were employed as variables for cluster and principal components analysis of these soybeans. The application of these multivariate methods enabled the grouping of the soybeans in different categories. The protein fractions obtained from these soybeans by the application of a fractionation method were also analyzed. The chromatographic profiles obtained enabled the assignment of peaks to the main soybean proteins (7S and 11S globulins). These data were used for the estimation, for the first time, of the 7S and 11S globulin contents in soybean cultivars.