Insights on Human Small Heat Shock Proteins and Their Alterations in Diseases.

The family of the human small Heat Shock Proteins (HSPBs) consists of ten members of chaperones (HSPB1-HSPB10), characterized by a low molecular weight and capable of dimerization and oligomerization forming large homo- or hetero-complexes. All HSPBs possess a highly conserved centrally located α-crystallin domain and poorly conserved N- and C-terminal domains. The main feature of HSPBs is to exert cytoprotective functions by preserving proteostasis, assuring the structural maintenance of the cytoskeleton and acting in response to cellular stresses and apoptosis. HSPBs take part in cell homeostasis by acting as holdases, which is the ability to interact with a substrate preventing its aggregation. In addition, HSPBs cooperate in substrates refolding driven by other chaperones or, alternatively, promote substrate routing to degradation. Notably, while some HSPBs are ubiquitously expressed, others show peculiar tissue-specific expression. Cardiac muscle, skeletal muscle and neurons show high expression levels for a wide variety of HSPBs. Indeed, most of the mutations identified in HSPBs are associated to cardiomyopathies, myopathies, and motor neuropathies. Instead, mutations in HSPB4 and HSPB5, which are also expressed in lens, have been associated with cataract. Mutations of HSPBs family members encompass base substitutions, insertions, and deletions, resulting in single amino acid substitutions or in the generation of truncated or elongated proteins. This review will provide an updated overview of disease-related mutations in HSPBs focusing on the structural and biochemical effects of mutations and their functional consequences.

Development of a Type I gluten-free sourdough.

UNLABELLED: The aim of this study was the setting up of a gluten-free sourdough from selected lactobacilli and yeasts isolated from a traditional wheat-based Type I sourdough. A gluten-free matrix was inoculated with Lactobacillus sanfranciscensis and Candida humilis, fermented to pH 4·0, and constantly propagated for ten times. A stable association between micro-organisms was observed from the second refreshment with mean values of 9·08 ± 0·25 log CFU g(-1) for lactobacilli and 7·81 ± 0·07 log CFU g(-1) for yeasts. In order to have a good workability of the dough, a 230 BU consistency was considered. Rheofermentographic indices remained constant over the ten refreshments, showing an average value of 23·2 mm dough height in about 7·5 h. The CO2 production and retention volumes reached average values of 1430 and 1238 ml respectively. The microbiological and technological data obtained highlighted that a GF sourdough was effectively developed. SIGNIFICANCE AND IMPACT OF THE STUDY: Type I sourdough has a long tradition as a leavening agent of baked goods as its use results in an improved texture, flavour, taste and extended shelf-life of the final products. In this study a Type I gluten-free sourdough was obtained. After few refreshments in controlled conditions, the sourdough presented a stable association between Lactobacillus sanfranciscensis and Candida humilis, constant fermentation times and technological properties (in terms of dough consistency, dough maximum height, CO2 production and retention). The results showed that the gluten-free sourdough developed in this study can improve the overall quality of gluten-free baked products.

Epidemiology of mouth cancer in 1989: a review.

The oral cavity and pharynx combined is the sixth commonest site of cancer in both sexes. In many countries the mortality rate is increasing among younger men born since approximately 1910-1920. A causal role in the aetiology of mouth cancer has been established for tobacco use, both smoking and chewing, separately and in conjunction with betel-quid chewing; with alcohol consumption and, less certainly, with other factors such as poor oral hygiene, nutritional factors and certain occupational exposures. In Western countries, there is convincing evidence that a large attributable risk can be ascribed to the joint habits of cigarette smoking and alcohol consumption. In Asian societies, a high attributable risk can be ascribed to cigarette smoking and betel chewing. Mouth cancer is at the same time an important form of cancer, and one for which practical prospects for prevention already exist. Against this background of a continually increasing trend among younger persons, it seems essential to engage upon programmes of prevention, including increasing awareness for early detection, against mouth cancer at the present time.