Eterogeneità delle proprietà fisico – chimiche della proteina prionica patologica e variabilità fenotipica ceppo specifica nella malattia di Creutzfeldt – Jakob

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are neurodegenerative disorders that affect humans and mammals. Creutzfeldt-Jakob disease (CJD), the most common TSE in humans, can be sporadic (sCJD), genetic (gCJD), or acquired by infection. All TSEs are characterised by the accumulation of PrPSc, a misfolded form of the cellular protein PrPC. PrPSc is insoluble in detergents, partially resistant to proteolysis and shows a highly enriched β-sheet secondary structure. Six clinico-pathological phenotypes of sCJD have been characterized which correlate at the molecular level with two types (1 or 2) of PrPSc with distinctive physicochemical properties and the genotype at the polymorphic (methionine or valine) codon 129 of the prion protein gene. According to the protein-only hypothesis, which postulates that prions are composed exclusively of PrPSc, the strains of prions that are largely responsible for the wide spectrum of TSE phenotypes are enciphered in PrPSc conformation. In support to this view, studies mainly conducted in experimental scrapie, have shown that several prion strains can be identified based on distinguishing PrPSc biochemical properties. To further contribute to the understanding of the molecular basis of strains and to develop more sensitive strain typing assays in humans we have analyzed PrPSc biochemical properties in two experimental setting. In the first we compared the size of the core after protease digestion and the glycoform pattern of PrPSc before and after transmission of human prions to non human primates or bank voles, whereas in the second we analyzed the conformational stability of PrPSc associated with sCJD, vCJD or fCJD using guanidine hydrochloride (GdnHCl) as denaturant. Combining the results of the two studies, we were able to distinguish five human strains for at least one biochemical property. The present data extend our knowledge about the extent of strain variation and its relationship with PrPSc properties in human TSEs.

Funzioni di Schur, jeu de Tacquin e regola di Littlewood-Richardson

Con l'obiettivo di fornire una dimostrazione della regola di Litlewood-Richardson, si forniscono nozioni su: composizioni e partizioni, funzioni quasisimmetriche, funzioni simmetriche, funzioni di Schur, e rappresentazioni lineari. Al termine sono inseriti un paio di ritratti dei due uomini che sono stati storicamente fondamentali per la trattazione degli argomenti della tesi.

Druze linguistic landscape in Israel: indexicality of new ethnolinguistic identity boundaries

The Druze community in Israel is a distinct religious community currently undergoing important ethnolinguistic shifts. The government's implementation of an official policy has led to the deconstruction and reshaping of the Druze political and national identity to one that differs substantially from that of the Palestinian minority in Israel. In this study, I argue that the visibility, vitality and appreciation of Hebrew in the Druze linguistic landscape are indicative of new ethnolinguistic boundaries of the Druze identity in Israel. The fact that the Druze in Israel are dispersed throughout the Galilee and Mount Carmel area and experience varying levels of language contact as well as divergent economic relations with their Palestinian–Israeli and Jewish–Israeli neighbors suggests that one cannot expect uniformity in the Druze linguistic markets or the processes of social, cultural and linguistic identification. This study will show that Hebrew has become a dominant component of the linguistic repertoire and social identity of the Druze in the Mount Carmel area since it has become the first choice of communication as the linguistic landscape indicates.

Does Israel Follow the Law? A Critical Analysis of Israel's Application of the International Law of Belligerent Occupation in the Occupied Territories

Israel's occupation of territories it captured in 1967 has become one of the longest and most controversial occupations of the last fifty years. Eschewing the traditional political analysis of the Israeli-Palestinian conflict, this paper aims to explore whether Israel has adequately applied international law in the occupied territories, in particular, the law of belligerent occupation. The two actors under assessment are the Israeli government, particularly its military which enforces and maintains the law in the territories, and the Supreme Court of Israel, which has the power of review over military actions in the territories. The particular issues of the occupation that are critically analyzed are the general legal framework that Israel established in the territories, Israel's civilian settlement policy in territories, and Israel's construction of a barrier in the West Bank. This paper concludes that Israel has incorrectly applied the legal framework of belligerent occupation by refusing to apply the Fourth Geneva Convention; it has wrongly concluded that the establishment of civilian settlements in the territories conform with international law; yet it has rightly concluded that the construction of the barrier in the West Bank is permissible under international law, in contrast to the conclusion of the much publicized International Court of Justice's Advisory Opinion on the 'Wall.' Along with these general assessments, the author will also provide some historical and political insight into why the Israeli government and the Supreme Court may have applied the law in the way that they did.