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The Machado-Joseph disease deubiquitylase ATX-3 couples longevity and proteostasis

K Kuhlbrodt, PC Janiesch, É Kevei, A Segref… - Nature Cell …, 2011 - nature.com
Protein ubiquitylation is a key post-translational control mechanism contributing to different physiological
processes, such as signal transduction and ageing. The size and linkage of a ubiquitin
chain, which determines whether a substrate is efficiently targeted for proteasomal ...
Cited by 6 - Related articles - All 3 versions

The Machado–Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability

TM Durcan, M Kontogiannea… - Human molecular …, 2011 - Oxford Univ Press
Machado–Joseph disease (MJD), the most common dominantly inherited ataxia worldwide, is
caused by a polyglutamine (polyQ) expansion in the deubiquitinating (DUB) enzyme
ataxin-3. Interestingly, MJD can present clinically with features of Parkinsonism. In this ...
Cited by 3 - Related articles - All 5 versions

Gabapentin for complex regional pain syndrome in Machado-Joseph disease: a case report

YC Lo, KK Liao, YC Lee… - Journal of Medical …, 2011 - jmedicalcasereports.com
A 29-year-old man (Han Chinese, Hoklo) with Machado-Joseph disease experienced severe
chronic pain in both feet, cutaneous thermal change, thermal hypersensitivity, focal edema, and
sweating and had a history of bone fracture. These symptoms are compatible with a ...
Cached - All 3 versions

Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado–Joseph disease

I Nascimento-Ferreira, T Santos-Ferreira… - Brain, 2011 - Oxford Univ Press
Machado–Joseph disease, also known as spinocerebellar ataxia type 3, is the most common
of the dominantly inherited ataxias worldwide and is characterized by mutant ataxin-3
misfolding, intracellular accumulation of aggregates and neuronal degeneration. Here we ...
Related articles - All 4 versions

Longitudinal study on MRI intensity changes of Machado–Joseph disease: correlation between MRI findings and neuropathological changes

Y Horimoto, M Matsumoto, H Akatsu, A Kojima… - Journal of …, 2011 - Springer
Abstract To disclose the neuropathological progression course of Machado–Joseph disease
(MJD), magnetic res- onance imaging (MRI) findings of six genetically con- firmed MJD cases
(four males and two females, including an autopsied female, all unrelated to one another) ...
Cited by 1 - Related articles - All 2 versions

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