نتایج جستجو برای: canavan
تعداد نتایج: 211 فیلتر نتایج به سال:
In a series of studies we examined sensory evoked potentials to stimuli presented while subjects performed a primary task. The primary tasks were designed to induce changes in slow cortical potentials (SCP) such as P300, contingent negative variation (CNV), and postimperative negative variation (PINV). Brief auditory stimuli were presented before, during or after the primary task. The interacti...
was a single case of true beriberi seen, whether inside the hospital or in the outpatients' department. Some cases showed ' dys-sebacia' and phrynoderma as a minor evidence of vitamin B or vitamin A deficiency, but more gross signs of avitaminosis such as cheilosis, glossitis, photophobia with corneal vascularization or pellagra were rare. A few cases, however, did have spongy gums and scattere...
Canavan's disease (CD) is a fatal pediatric leukodystrophy caused by mutations in aspartoacylase (AspA) gene. Currently, there is no effective treatment for CD; however, gene therapy is an attractive approach to ameliorate the disease. Here, we studied progressive neuropathology and gene therapy in short-lived (≤ 1 month) AspA(-/-) mice, a bona-fide animal model for the severest form of CD. Sin...
We show how Viennot’s combinatorial theory of orthogonal polynomials may be used to generalize some recent results of Sukumar and Hodges (Hodges & Sukumar 2007 Proc. R. Soc. A 463, 2401–2414 (doi:10.1098/rspa.2007.0001); Sukumar & Hodges 2007 Proc. R. Soc. A 463, 2415–2427 (doi:10.1098/rspa.2007.0003)) on the matrix entries in powers of certain operators in a representation of su(1, 1). Our res...
The extensive reform measures introduced in the early 90s in India provide a unique opportunity to study policy experiments aimed at reducing barriers to entry and increasing product market competition. We use a rich, comprehensive, plant-level dataset for the period 1986-87 to 1994-95 to identify the effects of de-licensing, liberalization of foreign direct investment (FDI) and the reduction o...
Predictive factors for the outcome of allogeneic transplantation in patients with MDS stratified according to the revised IPSS-R Matteo G. Della Porta, Emilio Paolo Alessandrino, Andrea Bacigalupo, Maria Teresa van Lint, Luca Malcovati, Cristiana Pascutto, Michele Falda, Massimo Bernardi, Francesco Onida, Stefano Guidi, Anna Paola Iori, Raffaella Cerretti, Paola Marenco, Pietro Pioltelli, Emanu...
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