Protein‐misfolding diseases and chaperone‐based therapeutic approaches

TK Chaudhuri, S Paul - The FEBS journal, 2006 - Wiley Online Library
A large number of neurodegenerative diseases in humans result from protein misfolding and
aggregation. Protein misfolding is believed to be the primary cause of Alzheimer's disease …

Histone deacetylase inhibitors as therapeutics for polyglutamine disorders

R Butler, GP Bates - Nature Reviews Neuroscience, 2006 - nature.com
During the past 5 years, gene expression studies in cell culture, animal models and in the
brains of patients have shown that the perturbation of transcription frequently results in …

Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates

X Zhong, RN Pittman - Human molecular genetics, 2006 - academic.oup.com
Expansion of a polyglutamine tract in ataxin-3 (AT3) results in spinocerebellar ataxia type
3/Machado–Joseph disease, one of the nine polyglutamine neurodegenerative diseases …

The two-stage pathway of ataxin-3 fibrillogenesis involves a polyglutamine-independent step

AM Ellisdon, B Thomas, SP Bottomley - Journal of Biological Chemistry, 2006 - ASBMB
The aggregation of ataxin-3 is associated with spinocerebellar ataxia type 3, which is
characterized by the formation of intraneuronal aggregates. However, the mechanism of …

Proteolytic cleavage of polyglutamine-expanded ataxin-3 is critical for aggregation and sequestration of non-expanded ataxin-3

A Haacke, SA Broadley, R Boteva… - Human molecular …, 2006 - academic.oup.com
Abstract Spinocerebellar ataxia type 3 (SCA3), like other polyglutamine (polyQ) diseases, is
characterized by the formation of intraneuronal inclusions, but the mechanism underlying …

Polyglutamine-expanded ataxin-3 activates mitochondrial apoptotic pathway by upregulating Bax and downregulating Bcl-xL

AH Chou, TH Yeh, YL Kuo, YC Kao, MJ Jou… - Neurobiology of …, 2006 - Elsevier
Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disease
caused by polyglutamine-expanded ataxin-3. In the present study, we expressed disease …

Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin‐3 immunopositive neuronal intranuclear inclusions

U Rüb, RAI De Vos, ER Brunt, T Sebestény… - Brain …, 2006 - Wiley Online Library
In the last years progress has been made regarding the involvement of the thalamus during
the course of the currently known polyglutamine diseases. Although recent studies have …

Mutant androgen receptor accumulation in spinal and bulbar muscular atrophy scrotal skin: a pathogenic marker

H Banno, H Adachi, M Katsuno, K Suzuki… - Annals of …, 2006 - Wiley Online Library
Objective Spinal and bulbar muscular atrophy (SBMA) is a hereditary motor neuron disease
caused by the expansion of a polyglutamine tract in the androgen receptor (AR). The …

Conformational diseases and ER stress-mediated cell death: apoptotic cell death and autophagic cell death

T Momoi - Current molecular medicine, 2006 - ingentaconnect.com
The expanded polyglutamine (polyQ) tracts observed in autosomal dominant
neurodegenerative disorders have the tendency to form intracellular aggregates, thus …

Imaging polyglutamine deposits in brain tissue

AP Osmand, V Berthelier, R Wetzel - Methods in enzymology, 2006 - Elsevier
The formation of polyglutamine aggregates occupies a central role in the pathophysiology of
neurodegenerative diseases caused by expanded trinucleotide repeats encoding the amino …