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Mutations in Surf1, a human gene involved in the assembly of cytochrome c oxidase (COX), cause Leigh syndrome, the most common infantile mitochondrial encephalopathy, characterized by a specific COX deficiency. We report the generation and characterization of functional knockdown (KD) lines for Surf1 in Drosophila. KD was produced by post-transcriptional silencing employing a transgene encoding a dsRNA fragment of the Drosophila homolog of human Surf1, activated by the UAS transcriptional activator. Two alternative drivers, Actin5C--GAL4 or elav--GAL4, were used to induce silencing ubiquitously or in the CNS, respectively. Actin5C--GAL4 KD produced 100% egg-to-adult lethality. Most individuals died as larvae, which were sluggish and small. The few larvae reaching the pupal stage died as early imagos. Electron microscopy of larval muscles showed severely altered mitochondria, elav--GAL4-driven KD individuals developed to adulthood, although cephalic sections revealed low COX-specific activity. Behavioral and electrophysiological abnormalities were detected, including reduced photoresponsiveness in KD larvae using either driver, reduced locomotor speed in Actin5C--GAL4 KD larvae, and impaired optomotor response as well as abnormal electroretinograms in elav--GAL4 KD flies. These results indicate important functions for SURF1 specifically related to COX activity and suggest a crucial role of mitochondrial energy pathways in organogenesis and CNS development and function.

The history of education cannot address the chapter of Italian Humanism by applying a sociological or psychosocial historiography that favors an abstract category of modernity. The problem of the expression of human virtues in civic life qualifies the Italian Quattrocento and helps to understand why the comparison with the Ancients generated a new sense of history precisely in that century. The “Civic Humanism” may be the theme that links a new desire for education with the innovation of the languages of an entire era. It is in this context that Eugenio Garin opened a new perspective onto the history of education through a review of Gentile’s historiography. He clarified the origins of modern Italy and the significance of the Italian fifteenth century for the development of European civilization. [ABSTRACT FROM AUTHOR]

The effects of [Cu.sup.2+], [Mn.sup.2+], [Zn.sup.2+] and [Al.sup.3+] in inducing defective conformational rearrangements of the recombinant human prion protein (hPrP), which trigger aggregation and fibrillogenesis, is investigated. The alteration of the variants of the 127-146 sequence has inhibited the fibrillogenesis process, indicating that integrity of the C-terminal region is needed both to confer amyloidogenic properties on Gerstmann-Straussler-Scheinker (GSS) peptides and to activate the stimulatory potential of the metal ions.

The Est-hetics of educating is not a new "special field" in philosophy, but an attempt to move away from the pedagogic experience of art towards a philosophy of experience that surpasses the limitations of a paideia entirely centred on the tenet of what man ought to be. The study of the concept of beauty, from the kalokagatia of the ancient Greeks, to the crisis of esthetic formalism in the 20th century, help us to understand that, to make pedagogy a science of life, it is necessary to move away from the disconnect between form and content and think again about the dialectic between critical thinking and that awareness of reality and beauty which is as much a part of our daily life as breathing. [ABSTRACT FROM AUTHOR]

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