Serine 776 of Ataxin-1 Is Critical for Polyglutamine-lnduced Disease in SCA Transgenic Mice;Interaction of Akt-Phosphorylated Ataxin-1 With 14-3-3 Mediates Neurodegeneration in Spinocerebellar Ataxia Type 1;Recovery From Polyglutamine-lnduced Neurode

摘要

Spinocerebellar ataxia type 1(SCA1)is an autosomal-dominant neurode-generative disease typically with midlife onset characterized by motor symptoms in the absence of cognitive deficits. The clinical features of SCA1 include ataxia,dysarthria,and swallowing and breathing problems. At the pathological level,the most frequent and severe alterations seen in SCA1 patients are the loss of Purkinje cells in the cerebellar cortex and degeneration of neurons in the inferior olivary nuclei,the cerebellar dentate nuclei,and the red nuclei.