2,691 research outputs found

    Lack of Mid1, the mouse ortholog of the Opitz syndrome gene, causes abnormal development of the anterior cerebellar vermis

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    Opitz G/BBB Syndrome (OS) is a genetic disorder characterized by midline developmental defects. Male patients with the X-linked form of OS, caused by loss-of-function mutations in the MID1 gene, show high variability of the clinical signs. MID1 encodes an ubiquitin ligase that controls Phosphatase 2A but its role in the pathogenesis of the disease is still unclear. Here I report a mouse line carrying a non-functional ortholog of the human MID1 gene, Mid1. Mid1 null mice show the brain anatomical defect observed in patients, i.e. hypoplasia of the anterior portion of the medial cerebellum, the vermis. We found that the presence of this defect correlates with motor coordination, procedural and non-associative learning impairments. The defect is limited to the most anterior lobes of the vermis, the region of the developing cerebellum adjacent to the dorsal midbrain. Analyses at mid-gestation reveal that lack of Mid1 causes the shortening of the posterior dorsal midbrain; the rostralization of the midbrain/cerebellum boundary; and the down-regulation of a key player in the development of this region, Fgf17. Thus, lack of Mid1 causes a mis-specification of the midbrain/cerebellar boundary that results in an abnormal development of the most anterior cerebellar lobes. This animal model provides a tool for further in vivo studies of the physiological and pathological role of the Mid1 gene and a system to investigate the development and function of anterior cerebellar domains

    Analisi, sviluppo e ottimizzazione di processi di trattamento del gas naturale mediante Aspen HYSYS.

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    Il gas naturale sta assumendo un ruolo sempre più importante nel panorama energetico e produttivo globale. In questo testo, le diverse fasi del trattamento del gas naturale, ed i diversi processi utilizzabili per realizzarle sono approfondite dapprima dal punto di vista teorico e successivamente mediante simulazioni realizzate con il software Aspen HYSYS. Lo studio si estende alle fasi di rimozione dei gas acidi, alla fase di disidratazione del gas e alla fase di rimozione/recupero degli NGL dal gas naturale, analizzando le molteplici configurazioni di processo precedentemente descritte dal punto di vista teorico. L'attenzione è rivolta non solo agli aspetti di analisi ed ottimizzazione delle diverse configurazioni di processo, ma anche ai possibili problemi impiantistici dei diversi processi di trattamento

    The variational theory of fracture: diffuse cohesive energy and elastic-plastic rupture

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    This communication anticipates some results of a work in progress [1], addressed to explore the efficiency of the diffuse cohesive energy model for describing the phenomena of fracture and yielding. A first local model is partially successful, but fails to reproduce the strain softening regime. A more robust non-local model, obtained by adding an energy term depending on the deformation gradient, describes many typical features of the inelastic response observed in experiments, including strain localization and necking. Fracture occurs as the result of extreme strain localization. The model predicts different fracture modes, brittle and ductile, depending on the analytical form of the cohesive energy function

    Tackling job insecurity : can a boundaryless career orientation boost job crafting strategies and career competencies?

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    none4siThe present study was aimed at investigating the mediating role played by job crafting and career competencies (i.e., knowing why, knowing how, and knowing whom) within the negative relation between boundaryless career orientation and job insecurity. A sample of 267 Italian employees fulfilled an online self-report questionnaire. Results of bootstrapping models with multiple mediators operating in serial indicated that boundaryless career orientation was negatively related to job insecurity through the subsequent mediation first of job crafting, and then of career competencies. This study provided support for the hypothesized relevance of training interventions focusing on the enhancement of a boundaryless perspective and job crafting strategies among HR best practices.mixedMazzetti, Greta; Lancioni, Chiara; Derous, Eva; Guglielmi, DinaMazzetti, Greta; Lancioni, Chiara; Derous, Eva; Guglielmi, Din

    Billy Elliot The Musical: visual representations of working-class masculinity and the all-singing, all-dancing bo[d]y

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    According to Cynthia Weber, ‘[d]ance is commonly thought of as liberating, transformative, empowering, transgressive, and even as dangerous’. Yet ballet as a masculine activity still remains a suspect phenomenon. This paper will challenge this claim in relation to Billy Elliot the Musical and its critical reception. The transformation of the visual representation of the human body on stage (from an ephemeral existence to a timeless work of art) will be discussed and analysed vis-a-vis the text and sub-texts of Stephen Daldry’s direction and Peter Darling’s choreography. The dynamics of working-class masculinity will be contextualised within the framework of the family, the older female, the community, the self and the act of dancing itself
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