89 research outputs found

    Avant-propos

    Get PDF
    En 2008 est paru en France un numéro de Pratiques sous la direction de Guy Achard-Bayle et Marie-Anne Paveau, intitulé Linguistique populaire ?, qui interroge pour la première fois de façon approfondie cette étiquette et le(s) domaine(s) de recherche qu’elle désigne. Le numéro s’appuie sur des travaux, rares en France à cette époque, portant sur l’activité métalinguistique des non-expert·e·s, sur le statut du sujet parlant et sur les questions épistémologiques qui s’ensuivent (Beacco 2004, Ho..

    120kev Ar8+-li Collisions Studied By Near Uv And Visible Photon Spectroscopy

    Get PDF
    A spectroscopic analysis of light emitted in the 200-600 nm wavelength range by Ar7+, Ar6+ and Ar5+ ions after charge exchange in 120keV Ar8+-Li collisions is performed. Transitions with Δn = 1 and Δn = 2 for n = 8, 9, 10 and 11 states of Ar8 following single electron capture are identified and the production cross sections for n = 8 and n = 9 are deduced from emission cross sections and compared with those calculated by the three-body classical trajectory Monte-Carlo method. Lines due to double capture process were observed and identified as Rydberg transitions 3snl-3sn\u27l\u27 (n = 7, 8 and 9) in Ar VII. Lines due to triple electron capture process were found and identified as transitions 3s2nl-3s2n\u27ï and 3s3pnl- 3s3pril\u27(n = 7, 8) in Ar VI. The configurations produced during the collision provides evidence that electron-electron interaction play an important role in double and triple charge exchange processes. © 1993 IOP Publishing Ltd

    LPIN1 gene mutations: a major cause of severe rhabdomyolysis in early childhood.

    Get PDF
    International audienceAutosomal recessive LPIN1 mutations have been recently described as a novel cause of rhabdomyolysis in a few families. The purpose of the study was to evaluate the prevalence of LPIN1 mutations in patients exhibiting severe episodes of rhabdomyolysis in infancy. After exclusion of primary fatty acid oxidation disorders, LPIN1 coding sequence was determined in genomic DNA and cDNA. Among the 29 patients studied, 17 (59%) carried recessive nonsense or frameshift mutations, or a large scale intragenic deletion. In these 17 patients, episodes of rhabdomyolysis occurred at a mean age of 21 months. Secondary defect of mitochondrial fatty oxidation or respiratory chain was found in skeletal muscle of two patients. The intragenic deletion, c.2295-866_2410-30del, was identified in 8/17 patients (47%), all Caucasians, and occurred on the background of a common haplotype, suggesting a founder effect. This deleted human LPIN1 form was unable to complement ∆pah1 yeast for growth on glycerol, in contrast to normal LPIN1. Since more than 50% of our series harboured LPIN1 mutations, LPIN1 should be regarded as a major cause of severe myoglobinuria in early childhood. The high frequency of the intragenic LPIN1 deletion should provide a valuable criterion for fast diagnosis, prior to muscle biopsy

    Les métadiscours des non-linguistes

    No full text
    International audienc
    corecore