Progetto e realizzazione di un Mini Aerial Vehicle a controllo semi-automatico per monitoraggio ambientale

Questa tesi si inserisce nel contesto del Progetto Si.Mon.A costituendone la prima tappa. Il lavoro di tesi riguarda la progettazione e realizzazione di una piattaforma di volo di piccole dimensioni per applicazioni di monitoraggio ambientale; si articola in tre parti. Nella parte prima si affronta il progetto concettuale con la concezione di un'architettura capace di soddisfare le specifiche, il suo successivo affinamento e l'approvazione del layout tramite studi di convalidazione aerodinamica, viene concepito uno schema per l'elettronica Fly By Wire di controllo e l'analisi del sistema propulsivo del velivolo. Nella seconda parte si affronta il progetto di dettaglio della cellula strutturale e di tutte le parti che la costituiscono, lo studio dell’elettronica a bordo destinata al controllo del volo e la scelta e caratterizzazione della propulsione fino ad arrivare al trittico dimensionato. Si affronta poi la costruzione materiale del prototipo, mettendo in evidenza i metodi usati e le tecniche di realizzazione impiegate. Nella terza parte si illustrano i risultati ottenuti con le prove in volo del velivolo

Preliminary Assessment of Radiolysis for the Cooling Water System in the Rotating Target of {SORGENTINA}-{RF}

The SORGENTINA-RF project aims at developing a 14 MeV fusion neutron source featuring an emission rate in the order of 5-7 x 10(13) s(-1). The plant relies on a metallic water-cooled rotating target and a deuterium (50%) and tritium (50%) ion beam. Beyond the main focus of medical radioisotope production, the source may represent a multi-purpose neutron facility by implementing a series of neutron-based techniques. Among the different engineering and technological issues to be addressed, the production of incondensable gases and corrosion product into the rotating target deserves a dedicated investigation. In this study, a preliminary analysis is carried out, considering the general layout of the target and the present choice of the target material

Genetic variants affecting NQO1 protein levels impact the efficacy of idebenone treatment in Leber hereditary optic neuropathy

Idebenone, the only approved treatment for Leber hereditary optic neuropathy (LHON), promotes recovery of visual function in up to 50% of patients, but we can neither predict nor understand the non -responders. Idebenone is reduced by the cytosolic NAD(P)H oxidoreductase I (NQO1) and directly shuttles electrons to respiratory complex III, bypassing complex I affected in LHON. We show here that two polymorphic variants drastically reduce NQO1 protein levels when homozygous or compound heterozygous. This hampers idebenone reduction. In its oxidized form, idebenone inhibits complex I, decreasing respiratory function in cells. By retrospectively analyzing a large cohort of idebenone-treated LHON patients, classified by their response to therapy, we show that patients with homozygous or compound heterozygous NQO1 variants have the poorest therapy response, particularly if carrying the m.3460G>A/MT-ND1 LHON mutation. These results suggest consideration of patient NQO1 genotype and mitochondrial DNA mutation in the context of idebenone therapy

L'analisi dei processi e l'implementazione del SIG in azienda: il caso Farmigea

Questa dissertazione ha ad oggetto sia una parte teorica che pratica, ossia un caso aziendale, riguardante l'analisi dei processi all'interno di un Gruppo aziendale e le successive fasi inerenti l'implementazione di un nuovo Sistema Informativo Gestionale, analizzando benefici attesi e criticità nascenti

Preliminary Assessment of Radiolysis for the Cooling Water System in the Rotating Target of SORGENTINA-RF

The SORGENTINA-RF project aims at developing a 14 MeV fusion neutron source featuring an emission rate in the order of 5–7 × 1013 s−1. The plant relies on a metallic water-cooled rotating target and a deuterium (50%) and tritium (50%) ion beam. Beyond the main focus of medical radioisotope production, the source may represent a multi-purpose neutron facility by implementing a series of neutron-based techniques. Among the different engineering and technological issues to be addressed, the production of incondensable gases and corrosion product into the rotating target deserves a dedicated investigation. In this study, a preliminary analysis is carried out, considering the general layout of the target and the present choice of the target material

SSBP1 mutations cause mtDNA depletion underlying a complex optic atrophy disorder

Inherited optic neuropathies include complex phenotypes, mostly driven by mitochondrial dysfunction. We report an optic atrophy spectrum disorder, including retinal macular dystrophy and kidney insufficiency leading to transplantation, associated with mitochondrial DNA (mtDNA) depletion without accumulation of multiple deletions. By whole-exome sequencing, we identified mutations affecting the mitochondrial single-strand binding protein (SSBP1) in 4 families with dominant and 1 with recessive inheritance. We show that SSBP1 mutations in patient-derived fibroblasts variably affect the amount of SSBP1 protein and alter multimer formation, but not the binding to ssDNA. SSBP1 mutations impaired mtDNA, nucleoids, and 7S-DNA amounts as well as mtDNA replication, affecting replisome machinery. The variable mtDNA depletion in cells was reflected in severity of mitochondrial dysfunction, including respiratory efficiency, OXPHOS subunits, and complex amount and assembly. mtDNA depletion and cytochrome c oxidase-negative cells were found ex vivo in biopsies of affected tissues, such as kidney and skeletal muscle. Reduced efficiency of mtDNA replication was also reproduced in vitro, confirming the pathogenic mechanism. Furthermore, ssbp1 suppression in zebrafish induced signs of nephropathy and reduced optic nerve size, the latter phenotype complemented by WT mRNA but not by SSBP1 mutant transcripts. This previously unrecognized disease of mtDNA maintenance implicates SSBP1 mutations as a cause of human pathology