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Is autophagy an elective strategy to protect neurons from dysregulated cholesterol metabolism?

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Neural Regeneration Research 2019年第4期14卷 582-587页

作者： Elisa Piscianz Liza Vecchi Brumatti Alberto tommasini Annalisa Marcuzzi Department of Medicine Surgery and Health Sciences University of Trieste Institute for Maternal and Child Health-IRCCS "Burlo Garofolo"

The balance of autophagy, apoptosis and necroptosis is crucial to determine the outcome of the cellular response to cholesterol dysregulation. Cholesterol plays a major role in regulating the properties of cell membranes, especially as regards their fluidity, and the regulation of its biosynthesis influences the shape and functions of these membranes. Whilst dietary cholesterol can easily be distributed to most organs, the central nervous system, whose membranes are particularly rich in cholesterol, mainly relies on de novo synthesis. For this reason, defects in the biosynthesis of cholesterol can variably affect the development of central nervous system. Moreover, defective synthesis of cholesterol and its intermediates may reflect both on structural cell anomalies and on the response to inflammatory stimuli. Examples of such disorders include mevalonate kinase deficiency, and Smith-Lemli-Opitz syndrome, due to deficiency in biosynthetic enzymes, and type C Niemann-Pick syndrome, due to altered cholesterol trafficking across cell compartments. Autophagy, as a crucial pathway dedicated to the degradation of cytosolic proteins and organelles, plays an essential role in the maintenance of homeostasis and in the turnover of the cytoplasmic material especially in the presence of imbalances such as those resulting from alteration of cholesterol metabolism. Manipulating the process of autophagy can offer possible strategies for improving neuronal cell viability and function in these genetic disorders.

关键词： cholesterol inflammation apoptosis autophagy neurons inherited disease necroptosis neuronal dysfunction

Genetics of inflammatory bowel disease from multifactorial to monogenic forms

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World Journal of Gastroenterology 2015年第43期21卷 12296-12310页

作者： Anna Monica Bianco Martina Girardelli Alberto tommasini Institute for Maternal and Child Health-IRCCS "Burlo Garofolo"

Inflammatory bowel disease(IBD) is a group of chronic multifactorial disorders. According to a recent study,the number of IBD association loci is increased to 201,of which 37 and 27 loci contribute specifically to the development of Crohn's disease and ulcerative colitis respectively. Some IBD associated genes are involved in innate immunity,in the autophagy and in the inflammatory response such as NOD2,ATG16L1 and IL23 R,while other are implicated in immune mediated disease(STAT3) and in susceptibility to mycobacterium infection(IL12B). In case of early onset of IBD(VEO-IBD) within the 6th year of age,the disease may be caused by mutations in genes responsible for severe monogenic disorders such as the primary immunodeficiency diseases. In this review we discuss how these monogenic disorders through different immune mechanisms can similarly be responsible of VEO-IBD phenotype. Moreover we would highlight how the identification of pathogenic genes by Next Generation Sequencing technologies can allow to obtain a rapid diagnosis and to apply specific therapies.

关键词： Inflammatory bowel disease Primary immunodeficienc

An important criterion for reliable multi-monochromatic x-ray imager diagnostics and its impact on the reconstructed images

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High Power Laser Science and Engineering 2015年第3期3卷 59-65页

作者： T.Nagayama R.C.Mancini D.Mayes R.tommasini R.Florido Physics Department University of Nevada Lawrence Livermore National Laboratory Departamento de Física Universidad de Las Palmas de Gran Canaria

Temperature and density asymmetry diagnosis is critical to advance inertial confinement fusion(ICF) science. A multimonochromatic x-ray imager, MMI, records the spectral signature from an ICF implosion core with time resolution, 2D spatial resolution and spectral resolution. While narrow-band images and 2D space-resolved spectra from the MMI data constrain the temperature and the density spatial structure of the core, the accuracy of the images and the spectra highly depends on the quality of the MMI data and the processing tools. Here, we synthetically investigate the criterion for reliable MMI diagnostics and its effects on the accuracy of the reconstructed images. The pinhole array tilt determines the object spatial sampling efficiency and the minimum reconstruction width, w. When the spectral width associated with w is significantly narrower than the spectral linewidth, the line images reconstructed from the MMI data become reliable. The MMI setup has to be optimized for every application to meet this criterion for reliable ICF diagnostics.

关键词： high power laser inertial confinement fusion monochromatic imager x-ray diagnostics

Control of wind-wave power on morphological shape of salt marsh margins

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Water Science and Engineering 2020年第1期13卷 45-56页

作者： Alvise Finotello Marco Marani Luca Carniello Mattia Pivato Marcella Roner Laura.tommasini Andrea D'alpaos Department of Geosciences University of PadovaPadova 35131Italy Department of Environmental Sciences Informatics and StatisticsUniversity of Venice Ca'FoscariVenice 30172Italy Center for Lagoon Hydrodynamics and Morphodynamics University of PadovaPadova 35131Italy Department of Civil Environmental and Architectural EngineeringUniversity of PadovaPadova 35131Italy

Salt marshes are among the most common morphological features found in tidal landscapes and provide ecosystem services of primary ecological and economic ***,the continued rise in relative sea level and increasing anthropogenic pressures threaten the sustainability of these *** alarmingly high rates of salt marsh loss observed worldwide,mainly dictated by the lateral erosion of their margins,call for new insights into the mutual feedbacks among physical,biological,and morphological processes that take place at the critical interface between salt marshes and the adjoining tidal *** combined field measurements,remote sensing data,and numerical modeling to investigate the interplays between wind waves and the morphology,ecology,and planform evolution of salt marsh margins in the Venice Lagoon of *** results confirm the existence of a positive linear relationship between incoming wave power density and rates of salt marsh lateral *** addition,we show that lateral erosion significantly decreases when halophytic vegetation colonizes the marsh margins,and that different erosion rates in vegetated margins are associated with different *** marsh cliffs and smooth shorelines are expected along rapidly eroding margins,whereas erosion rates are reduced in gently sloped,irregular edges facing shallow tidal flats that are typically exposed to low wind-energy *** highlighting the relationships between the dynamics and functional forms of salt marsh margins,our results represent a critical step to address issues related to conservation and restoration of salt marsh ecosystems,especially in the face of changing environmental forcings.

关键词： Salt marshes Wind waves Lateral erosion Morphodynamics Vegetation

Druggable monogenic immune defects hidden in diverse medical specialties:Focus on overlap syndromes

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World Journal of Clinical Pediatrics 2022年第2期11卷 136-150页

作者： Valentina Boz Chiara Zanchi Laura Levantino Guglielmo Riccio Alberto tommasini Department of Medical Surgical and Health SciencesUniversity of TriesteTrieste 34137Italy Department of Pediatrics Institute for Maternal and Child HealthIRCCS Burlo GarofoloTrieste 34137Italy

In the last two decades two new paradigms changed our way of perceiving primary immunodeficiencies:An increasing number of immune defects are more associated with inflammatory or autoimmune features rather than with *** primary immune defects are due to hyperactive pathways that can be targeted by specific inhibitors,providing innovative precision treatments that can change the natural history of *** this article we review some of these“druggable”inborn errors of immunity and describe how they can be suspected and diagnosed in diverse pediatric and adult medicine *** the availability of precision treatments can dramatically impact the course of these diseases,preventing the development of organ damage,it is crucial to widen the awareness of these conditions and to provide practical hints for a prompt detection and cure.

关键词： Inborn errors of immunity Primary immunodeficiency diseases Precision treatments Immunodysregulation Autoimmunity Overlap syndromes

Ages of celiac disease: From changing environment to improved diagnostics

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World Journal of Gastroenterology 2011年第32期17卷 3665-3671页

作者： Alberto tommasini Tarcisio Not Alessandro Ventura Laboratory of Immunopathology Institute for Maternal and Child Health Scientific Institute for Research and Care Burlo Garofolo and University of Trieste Department of Pediatrics Institute for Maternal and Child Health Scientific Institute for Research and Care Burlo Garofolo and University of Trieste

From the time of Gee's landmark writings, the recent history of celiac disease (CD) can be divided into many ages, each driven by a diagnostic advance and a deeper knowledge of disease pathogenesis. At the same time, these advances were paralleled by the identification of new clinical patterns associated with CD and by a continuous redefinition of the prevalence of the disease in population. In the beginning, CD was considered a chronic indigestion, even if the causative food was not known; later, the disease was proven to depend on an intolerance to wheat gliadin, leading to typical mucosal changes in the gut and to a malabsorption syndrome. This knowledge led to curing the disease with a gluten-free diet. After the identification of antibodies to gluten (AGA) in the serum of patients and the identification of gluten-specific lymphocytes in the mucosa, CD was described as an immune disorder, resembling a chronic "gluten infection". The use of serological testing for AGA allowed identification of the higher prevalence of this disorder, revealing atypical patterns of presentation. More recently, the characterization of autoantibodies to endomysium and to transglutaminase shifted the attention to a complex autoimmune pathogenesis and to the increased risk of developing autoimmune disorders in untreated CD. New diagnostic assays, based on molecular technologies, will introduce new changes, with the promise of better defining the spectrum of gluten reactivity and the real burden of gluten related-disorders in the population. Herein, we describe the different periods of CD experience, and further developments for the next celiac age will be proposed.

关键词： Antibodies Autoimmunity Celiac disease Diagnostics History Intestinal mucosa

Chinese visiting Rovaniemi, Finland: profile and perceptions of Arctic tourists

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Advances in Polar Science 2016年第3期27卷 200-208页

作者： Daniela.tommasini Shenghan Zhou Arctic Centre University of Lapland Multidimensional Tourism Institute University of Lapland

The profile of the Chinese tourist is changing. Outbound tourism destinations for Chinese tourists are no longer limited to the Asia-Pacific region. Chinese tourists visit many different destinations, and the areas around the Arctic Circle are growing in attraction. This paper is based on a 2016 online survey about Chinese tourists visiting Rovaniemi, Finland. The questions were based on a previous study of the Finnish Tourist Board conducted in 2003. The results were analyzed and the two studies were compared. The resultant profile of the Chinese tourist visiting the Rovaniemi area showed natural landscapes to be the most important attraction. All respondents had a ＂must-do＂ activity, and more than half participated in sport and leisure activities. The state of the environment, natural landscape, and safety conditions were rated most important among tourists. Increasingly, tourists prefer to organize individual trips, using multiple information channels. Currently, Chinese tourists demand deeper tourism experiences. They want to enjoy winter activities in a pure environment, and unique experiences, such as sleeping in an ice or glass hotel. Chinese tourists appreciate the uniqueness that cannot be found in other places. They wish to benefit from travel information from various media, which will allow Chinese tourists to make a complete plan before their journey.

关键词： Chinese outbound tourists Arctic tourist profile tourist perceptions

Altered pattern of tumor necrosis factor-alpha production in peripheral blood monocytes from Crohn's disease

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World Journal of Gastroenterology 2016年第41期22卷 9117-9126页

作者： Claudia Loganes Alessia Pin Samuele Naviglio Martina Girardelli Anna Monica Bianco Stefano Martelossi Alberto tommasini Elisa Piscianz Department of Medicine Surgery and Health Sciences University of Trieste Department of Pediatrics Institute for Maternal and Child Health IRCCS "Burlo Garofolo"

AIM To evaluate the inflammatory state in Crohn's disease(CD) patients and correlate it with genetic background and microbial *** By means of flow cytometry, production of tumor necrosis factor-alpha(TNF-α) was measured in peripheral blood monocytes from patients suffering from CD, ulcerative colitis(UC) and in healthy subjects after stimulation of the NOD2 and TLR pathways. CD patients were genotyped for the three most common NOD2 variants(R702W, G908 R and L1007Pfs*2) and basal production of TNF-α was correlated to NOD2 genotype. Also, production of TNF-α was correlated to plasmatic levels of LPS Binding Protein(LBP), soluble(s) CD14 and to the activity state of the *** The patients with CD were characterized by a significantly higher monocyte basal expression of TNF-αcompared with healthy subjects and UC patients, and after stimulation with Pam3CSK4(ligand of TLR2/1) and MDP-L18(ligand of NOD2) this difference was maintained, while other microbial stimuli(LPS, ligand of TLR4 and Poly I:C, ligand of TLR3) induced massive activation in CD monocytes as well as in UC and in healthy control cells. There was no significant difference in the production of TNF- α between patients who carried CD-associated heterozygous or homozygous variants in NOD2 and patients with wild type NOD2 genotype. Although serum LBP levels have been shown to correlate positively with the state of activity of the disease, TNF-α production did not show a clear correlation with either LBP or s CD14 levels in plasma. Moreover, no clear correlation was seen between TNF-α production and activity indices in either CD or *** Peripheral monocytes from CD express higher basal and stimulated TNF-α than controls, regardless of NOD2 genotype and without a clear correlation with disease activity.

关键词： Crohn’s disease Ulcerative colitis Tumor necrosis factor-α NOD2 variants Toll like receptors Dysbiosis Activity index LPS-binding protein

Ex vivo response to mucosal bacteria and muramyl dipeptide in inflammatory bowel disease

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World Journal of Gastroenterology 2016年第44期22卷 9734-9743页

作者： Claudia Loganes Erica Valencic Alessia Pin Elisa Marini Stefano Martelossi Samuele Naviglio Luigina De Leo Tarcisio Not Lorenzo Monasta Alberto tommasini Annalisa Marcuzzi Department of Medicine Surgery and Health Sciences University of Trieste Strada di Fiume Institute for Maternal and Child Health - IRCCS "Burlo Garofolo"

AIM To evaluate how mucosal bacteria impact on the spontaneous and muramyl dipeptide(MDP)-induced inflammation in Crohn's disease(CD) and ulcerative colitis(UC).METHODS Colonic mucosal biopsies were collected from children with active or remissive CD, UC and controls. Two tissue samples were taken from inflamed mucosal segments(in patients with active disease) or from noninflamed mucosa [in patients in remission or in healthy controls(HC)]. Experiments were performed in the presence or absence of antibiotics, to assess whether the disease-associated microbiota can modulate the cytokine response ex vivo. For this purpose, each specimen was half-cut to compare spontaneous and MDP-induced inflammation in the presence of live bacteria(LB) or antibiotics. After 24 h of culture, an array of 17 cytokines was assessed in supernatants. Statistical analyses were performed to find significant differences in single cytokines or in patterns of cytokine response in the different groups. RESULTS We demonstrated that subjects with CD display a spontaneous production of inflammatory cytokines including granulocyte-colony stimulating factor(G-CSF), interleukin(IL) 6, IL8, IL10 and IL12, that was not significantly influenced by the addition of antibiotics. UC specimens also displayed a trend of increased spontaneous secretion of several cytokines, which however was not significant due to broader variability among patients. After the addition of antibiotics, spontaneous IL8 secretion was significantly higher in UC than in controls. In HC, a trend towards the weakening of spontaneous IL8 production was observed in the presence of live mucosal bacteria with respect to the presence of antibiotics. In contrast, in the presence of LB UC showed an increasing trend of spontaneous IL8 production, while MDP stimulation resulted in lower IL8 production in the presence of antibiotics. We also showed that subjects with CD seem to have a lowered production of IL8 in response to MDP in the presence

关键词： Gut-microbiota Crohn’s disease Ulcerative colitis Cytokines Inflammation