Sechi, Gianpietro and Matta, Manuela and Deiana, Giovanni Andrea and Balbi, Pietro and Bachetti, Tiziana and Di Zanni, Eleonora and Ceccherini, Isabella and Serra, Alessandro (2010) Ceftriaxone has a therapeutic role in Alexander disease. Progress in Neuro-Psychopharmacology and Biological Psychiatry, Vol. 34 (2), p. 416-417. eISSN 1878-4216. Article.
Full text not available from this repository.
Alexander disease (AD) (MIM 203450) is a rare, usually fatal neurodegenerative disorder, involving primarily astroglial cells in the CNS, caused by dominant mutations in the gene encoding glial fibrillary acidic protein (GFAP) (Brenner et al., 2001). It is characterized by dystrophic astrocytes containing intermediate filament aggregates (Rosenthal fibers) (RFs), in combination with myelin abnormalities (Li et al., 2005). Pathogenetic determinants include a toxic gain-of-function of mutated GFAP which causes aggregates and RFs accumulation in astrocytes and an excitotoxicity related to impairment of the buffering capacity of dystrophic astrocytes and of their ability to metabolize extracellular glutamate ([Mignot et al., 2004] and [Sullivan et al., 2007]).
I documenti depositati in UnissResearch sono protetti dalle leggi che regolano il diritto d'autore
Repository Staff Only: item control page