Tandem repeat diseases include the neurodegenerative disorders known as polyglutamine
(polyQ) diseases, caused by CAG repeat expansions in the coding regions of the respective …

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 …

Disease-associated misfolded proteins or proteins damaged due to cellular stress are
generally disposed via the cellular protein quality-control system. However, under saturating …

The most frequent subtype of autosomal dominant inherited spinocerebellar ataxias is
caused by CAG repeat expansions of more than 55 units in the ataxin-3 gene. The clinical …

Abstract Machado-Joseph Disease (MJD), also known as spinocerebellar ataxia type 3
(SCA3), represents the most common form of SCA worldwide. MJD is an autosomal …

The object of this review is to assemble much of the literature concerning Purkinje cell death
in cerebellar pathology and to relate this to what is now known about the complex …

A hallmark of most neurodegenerative diseases, including those caused by polyglutamine
expansion, is the formation of ubiquitin (Ub)-positive protein aggregates in affected neurons …

Machado–Joseph disease (MJD; MIM 109150) is a late-onset neurodegenerative disorder
caused by the expansion of a polyglutamine tract within the MJD1 gene. We have previously …

CAG-repeat expansions in at least eight different genes cause neurodegeneration. The
length of the extended polyglutamine stretches in the corresponding proteins is …