The reserch progress in the pathogenesis of Spinocerebellar Ataxia Type 3

Abstract

Spinocerebellar Ataxia Type 3 / Machado-Joseph Disease (SCA3/MJD) is an autosomal dominantly inherited late-onset neurodegenerative disorder,with high morbidity and high mortality.This disease is a serious threat to the health and quality of life for SCD3/MJD patients.The exact pathogenesis of this disease is still poorly understood.Also,effective trcatment for SCD3 is lacking in clinics.The current domestic and international research shows that a number of factors related to this disease such as disease genes,pathogenic proteins,neuronal intranuclear inclusions,ubiquitin-proteasome pathway,autophagy-lysosomal pathway,gene transcription,chaperones,mitochondrial oxidative stress,apoptosis and many other factors.In this paper,we make a brief review about the research progress of the pathogenesis for the disorder. Key words: Spinocerebellar ataxia type 3; Machado-Joseph disease; SCA3/ MJD; Pathogenesis