Impairment of blood-brain barrier is an early event in R6/2 mouse model of Huntington Disease

• DOI: 10.1038/srep41316
• Publication Date: 2017-01-24T11:11:19Z
• Authors: Alba Di Pardo; Enrico Amico; Francesco Scalabrì; Giuseppe Pepe; Salvatore Castaldo; Francesca Elifani; Luca Capocci; Claudia De Sanctis; Laura Comerci; Francesco Pompeo; Maurizio D’Esposito; Stefania Filosa; Stefania Crispi; Vittorio Maglione
• Keywords: Brain Function

Abstract Blood-brain barrier (BBB) breakdown, due to the concomitant disruption of tight junctions (TJs), normally required for maintenance BBB function, and altered transport molecules between blood brain vice-versa, has been suggested significantly contribute development progression different disorders including Huntington’s disease (HD). Although detrimental consequence breakdown may have in clinical settings, timing its alteration remains elusive many neurodegenerative diseases. In this study we demonstrate first time that HD is not confined established symptoms, but occurs early progression. Despite obvious signs impaired permeability were only detectable concomitance with onset disease, deranged TJs integrity occur precociously precede overt symptoms. To our perspective finding add a new dimension horizons pathological mechanisms underlying devastating however much be elucidated understanding how specific drug targets can approached future.