T he stability of the proteome is crucial to the health of the cell, and contributes significantly
to the lifespan of the organism. Aging and many age-related diseases have in common the …

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 …

Abstract Machado‐Joseph disease (MJD)/spinocerebellar ataxia type 3 (SCA3) is an
autosomal dominant neurodegenerative disorder caused by polyglutamine expansion in the …

Transcriptional dysregulation has been described in spinocerebellar ataxia type 3/Machado-
Joseph disease (SCA3/MJD), an autosomal dominant ataxia caused by a polyglutamine …

Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disorder, characterised by
progressive motor neuron degeneration and muscle paralysis. Heat shock proteins (HSPs) …

Machado–Joseph disease (MJD, also known as spinocerebellar ataxia type 3, SCA3), an
autosomal dominant neurological disorder, is caused by an abnormal expanded …

The catalytically inactive isoforms of α-carbonic anhydrases are known as carbonic
anhydrase related proteins (CARPs). The CARPs occur independently or as domains of …

We have investigated the expression of Hsp25, a heat shock protein constitutively
expressed in motoneurons, in amyotrophic lateral sclerosis (ALS) mice that express G93A …

Polyglutamine (polyQ)-expanded mutant ataxin-3 protein, which is prone to misfolding and
aggregation, leads to cerebellar neurotoxicity in spinocerebellar ataxia type 3 (SCA3), an …

Purpose To measure the cytoprotective effects of rhHsp70 against oxidative stress and study
its cellular uptake, intracellular and intraocular distribution in the retinal pigment epithelium …