121,868 research outputs found
Papillon-Lefevre Syndrome
No full textPapillon-Lefevre syndrome (PLS) is a rare autosomal recessive disorder characterized by severe early-onset periodontal destruction involving both the deciduous and permanent dentition and skin lesions of palmoplantar hyperkeratosis. Recently it was found that PLS patients carry loss-of-function mutations in the gene encoding cathepsin C (CTSC). The aim of this study was analyzed the CTST gene in one Czech family with PLS. Peripheral blood samples were obtained from individuals belonging to this family for DNA isolation. Mutations were identified by direct sequencing of genomic DNA amplified for selected regions of the cathepsin C gene. 12-old year patient and his grandfather had a classic PLS phenotype. We analyzed all exons of the CTSC gene and identified a mutation in intron 1 (rs217077). Both affected individuals were homozygous for T allele; our child's mother and unaffected brother carried a heterozygote combination of this mutation. Dipeptidyl-peptidase I encoded by the CTSC gene play an immune and inflammatory role. Many different mutations in this gene have been reported in patients with PLS from several ethnic groups. This is the first genetic study of Papillon-Lefevre syndrome in the Czech population
La Boderie, Guy Lefevre de
No full textGuy Lefevre de La Boderie was a renowned orientalist philologist, a translator of ancient and modern languages, an expert in mystical and especially cabalistic doctrines, and a poet. His works were crucial to the spread of Neoplatonic and cabalistic motifs within French Renaissance culture. Influenced by Guillaume Postel, he fostered peaceful dialogue and gradual integration of all religious creeds in light of a cabalistic interpretation of Catholicism
Una poesia per l’Impero : Lingua, editoria e pratica del petrarchismo tra Spagna e Italia nell’epoca di Carlo V
No full textIn questo ampio studio monografico si traccia un profilo storico, linguistico e letterario di quella che fu l?epoca della penetrazione della poesia italiana nella civiltà letteraria della Spagna di Carlo V. Diversamente dai molti classici studi sul petrarchismo spagnolo e italiano, che hanno lavorato su un unico versante delle due realtà, la
novità principale, e sostanziale, della monografia risiede nell'individuazione di un unico sistema di riferimento politico, culturale e linguistico-letterario ? l?Impero di Carlo d'Asburgo che coinvolge le forme e i contenuti della poesia lirica di entrambi i paesi. Soprattutto attraverso l'ampliamento del mercato tipografico all'interno dei confini imperiali, dalla penisola iberica a quella italiana ai vari domini spagnoli in Europa e nel Nuovo Mondo, si diffonde, da un lato, lo stile di vita fissato dal Cortegiano di Castiglione, presto tradotto in Spagna insieme al suo vasto apparato di regole e precetti, dall'altro, il generale codice del petrarchismo lirico, con tutte le sue implicazioni di natura sociale, linguistica e ideologica
de Moreau de Gerbehaye (Claude). L 'abrogation des privileges fiscaux et ses antécédents. La lente maturation du cadastre thérésien au duché de Luxembourg (1684- 1774)
No full textDouxchamps-Lefevre Cécile. de Moreau de Gerbehaye (Claude). L 'abrogation des privileges fiscaux et ses antécédents. La lente maturation du cadastre thérésien au duché de Luxembourg (1684- 1774). In: Revue belge de philologie et d'histoire, tome 76, fasc. 2, 1998. Histoire medievale, moderne et contemporaine - Middeleeuwse, moderne en hedendaagse geschiedenis. pp. 629-631
Stelle distanti. Una nota sulle traduzioni italiane di Pío Baroja
No full textL'articolo analizza in prospettiva diacronica le traduzioni italiane di Pio Baroja attraverso il Novecento e i primi anni Duemila
Jeanne L. Hauser, 99
No full textJeanne LeFevre Hauser, who lived in Palo Alto and Portola Valley, has died. She was 99. Jeanne, who died on February 28, was born on June 23, 1921
Going Beyond Counting First Authors in Author Co-citation Analysis
No full textThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Square Dancing with the Stars to Enhance Dynamic Hirschman Linkages?
No full textIn this Presidential Address, the author takes the reader on a reconnaissance of his life and time as a regional scientist. He points out scenery he found scintillating along the way, hoping that some may pick up the banner and chew on a few of the ideas for a while. He suggests a revisit to Albert O. Hirschman’s notion of key sectors and more empirical analysis related to Marcus Berliant’s and Masahisa Fujita’s notion of knowledge creation and transfer.Presidential Address, San Antonio, Texas, March 29, 2014 (53rd Meetings of the Southern Regional Science Association
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