Heat shock proteins (HSPs) are induced in response to many injuries including stroke,
neurodegenerative disease, epilepsy, and trauma. The overexpression of one HSP in …

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 …

Polyglutamine (polyQ) diseases are a group of nine neurodegenerative disorders caused by
an unstable CAG expansion in the codifying region of their respective associated genes …

Expansion of a polyglutamine tract in ataxin-3 (polyQ) causes Machado–Joseph disease, a
late-onset neurodegenerative disorder characterized by ubiquitin-positive aggregate …

Protein aggregation is under intense scrutiny because of its role in human disease. Although
increasing evidence indicates that protein native states are highly protected against …

Mutant ataxin-3 is aberrantly folded and proteolytically cleaved in spinocerebellar ataxia
type 3. The C-terminal region of the protein includes a polyglutamine stretch that is …

We focus on hepatic inclusions, which are defined as intracellular aggregates of stainable
substances. They represent established hallmarks of their respective human disorders, but …

Neurotrophic Factors............................................................................................... 38 Cytoskeletal
Defects and Axonal Transport............................................................. 39 A Role for the Loss of …

Dentatorubral-pallidoluysian atrophy (DRPLA) is caused by the expansion of polyglutamine
(polyQ) in atrophin-1 (ATN1), also known as DRPLA protein. ATN1 is ubiquitously …

To disclose the neuropathological progression course of Machado–Joseph disease (MJD),
magnetic resonance imaging (MRI) findings of six genetically confirmed MJD cases (four …