The spinocerebellar ataxias (SCAs) comprise more than 40 autosomal dominant
neurodegenerative disorders that present principally with progressive ataxia. Within the past …

Heat shock protein 70 (Hsp70a) is a molecular chaperone that is expressed in response to
stress. In this role, Hsp70 binds to its protein substrates and stabilize them against …

Machado–Joseph disease (MJD; also called spinocerebellar ataxia type 3) is a dominantly
inherited late-onset neurodegenerative disorder caused by expansion of polyglutamine …

Protein toxicity can be defined as all the pathological changes that ensue from
accumulation, mis-localization, and/or multimerization of disease-specific proteins. Most …

Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is
the most common inherited spinocerebellar ataxia and one of many polyglutamine …

Abstract Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3
(SCA 3), is an incurable disorder, widely regarded as the most common form of …

Slowly progressive ataxia accompanied by cerebellar degeneration is often genetic in
origin. The past 15 years have witnessed a revolution in our understanding of the causes of …

Control of proper protein synthesis, function, and turnover is essential for the health of all
cells. In neurons these demands take on the additional importance of supporting and …

Purpose This study aimed to unravel the genetic factors underlying missing heritability in
spinocerebellar ataxia type 17 (SCA17) caused by polyglutamine-encoding CAG/CAA …

Expansion of a polyglutamine tract in the androgen receptor (AR) causes spinal and bulbar
muscular atrophy (SBMA). We previously showed that Akt-mediated phosphorylation of AR …