430 research outputs found

    Crisis of Parties and Change of Party System in Italy

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    Leonardo Morlino. Is there a crisis of democracy in Southern Europe? 30 cm. This paper is based partly on a seminar entitled Is there a crisis of democracy in Southern Europe?" presented at the Center on November 16, 1995" - T.p. Includes bibliographical references (p. 30-32

    Conclusioni. I risultati della ricerca

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    Il saggio tira le fila dei vari contributi raccolti nel volume, riconducendo i principali risultati della ricerca empirica alle trasformazioni indotte dall'europeizzazione su quattro livelli della rappresentanza: semplice/composita; funzionale/territoriale; elettiva/non elettiva; identità/interessi

    Cosmic ray electrons released by supernova remnants

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    The process that allows cosmic rays (CRs) to escape from their sources and be released into the Galaxy is still largely unknown. The comparison between CR electron and proton spectra measured at Earth suggests that electrons are released with a spectrum steeper than protons by Δsep 0.3 for energies above 10 GeV and by Δsep 1.2 above 1 TeV. Assuming that both species are accelerated at supernova remnant shocks, we here explore two possible scenarios that can in principle justify steeper electron spectra: (i) energy losses due to synchrotron radiation in an amplified magnetic field and (ii) time-dependent acceleration efficiency. We account for magnetic field amplification produced by either CR-induced instabilities or by magnetohydrodynamics instabilities my means of a parametric description. We show that both mechanisms are required to explain the electron spectrum. In particular, synchrotron losses can only produce a significant electron steepening above 1 TeV, while a time-dependent acceleration can explain the spectrum at lower energies if the electron injection into diffusive shock acceleration is inversely proportional to the shock speed. We discuss observational and theoretical evidences supporting such a behaviour. Furtheore, we predict two additional spectral features: a spectral break below few GeV (as required by existing observations) due to the acceleration efficiency drop during the adiabatic phase, and a spectral hardening above 20 TeV (where no data are available yet) resulting from electrons escaping from the shock precursor

    Changes for Democracy: Actors, Structures, Processes

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    In this major new work, the author presents a broad-ranging and far-reaching analysis of the process of democratization. Having reviewed the main theoretical positions in the literature, he goes on to propose a new definition of democracy and to highlight and discuss the existence of a new type of regime: the hybrid regime. The second and third parts of the book cover three geopolitical areas (Southern Europe, Eastern Europe, and Latin America) and examine the main issues in the process of democratization across each area, including the transition toward democracy, installation, consolidation and crisis. From this analysis a new, more nuanced theoretical position is developed and presented. The analysis provides the richest reflection yet on one of the most important political processes of the modern times. The book will be essential reading for all scholars and students of democratization

    Hereditary connective tissue disorders: novel genotype-phenotype correlations, disease-genes and pathogenetic factors affecting disability

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    Hereditary soft connective tissue disorders (HCTDs)are a group of clinically variable and genetically heterogeneous conditions caused by constitutional abnormalities of the various components of the extracellular matrix (ECM). Although singularly rare, this community of syndromes are an emerging clinical phenomenon commonly encountered in different specialized settings, including but not limited to rheumatology, physical medicine, cardiology/heart surgery, and clinical genetics. EDS is probably the most common reason of referral in outpatient services dedicated to HCTDs. The 2017 international classification identifies 13 different clinical EDS forms due to mutations in no less than 19 genes. Nevertheless, probably no more than 20% of the patients who have a clinical diagnosis of EDS receive a molecular confirmation by the identification of the responsible mutation. On the other hand, EDS shares many features with the other HCTDs, and such a clinical similarity likely mirrors a common pathogenesis, at least, in specific cases. Therefore, it is expected that novel genes and novel genotype-phenotype correlations for known genes will be identified for EDS. Understanding the biological variability underpinning such a clinical variability is of utmost importance for seeking a tailored approach in the management of these patients. San Camillo-Forlanini Hospital (SCFH) is the Coordinator of the Regional (Lazio, Italy) reference centers for EDS and full member of the European Reference Network (ERN) for rare musculoskeletal diseases (ReCONNET). Since 2010, more than 800 patients who were referred to SCFH, received a diagnosis of EDS and were put under periodic medical follow-up. The aims of this PhD project were: 1) to highlight new genotype-phenotype correlations in HCTDs; 2) to identify novel disease genes for EDS-like phenotypes; 3) to explore disability determinants in a EDS patient cohort. In this context, for the years 2017-2019, the results of the current PhD project included (i) the discovery of the molecular cause of the exceptionally rare X-linked EDS variant, which resulted mutated in FLNA; (ii) the identification of TAB2 and MAP3K7, two genes encoding components of the non-canonic TGFβ-pathway, as responsible of phenotypes resembling EDS; (iii) characterization of musculoskeletal phenotype of hypermobile EDS, the most common EDS variant; (iv) characterization of the skin phenotype of classical EDS, the second most common EDS variant; (v) the exploration of the neurodevelopmental attributes of joint hypermobility in children with different forms of EDS and adults with hEDS in order to open the path for the identification of severity scoring in these disorders; (vi) the delineation of a severity scoring for adults with hypermobile EDS and HSD (hEDS/HSD); (vii) the clinical and molecular characterization of osteogenesis imperfecta/EDS overlap (OI/EDS); and (viii) the exploration of cellular effects of the mutations identified in TAB2 and MAP3K7. The related research activity resulted in 15 publications and SCFH was also represented in the International committee who published the current classification on EDS. The project has been nurtured by a multispecialistic team, aimed at exploring the biological and clinical variability of HCTDs with a true translational intent
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