Results 191 to 200 of about 21,037 (206)

Polyglutamine conformation in GST-polyglutamine fusion proteins [PDF]

open access: possibleBiochemical Society Transactions, 2002
Annalisa Pastore   +4 more
openaire   +1 more source
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Therapeutic opportunities in polyglutamine disease

Nature Medicine, 2001
Polyglutamine diseases comprise a class of familial neurodegenerative disorders caused by expression of proteins containing expanded polyglutamine tracts. Great progress has been made in elucidating the molecular mechanisms contributing to polyglutamine pathology, and in identifying potential drug targets.
Robert E. Hughes, James M. Olson
openaire   +3 more sources

Role of proteolysis in polyglutamine disorders

Journal of Neuroscience Research, 2003
AbstractTo date, nine polyglutamine disorders have been characterised, including Huntington's disease (HD), spinobulbar muscular atrophy (SBMA), dentatorubral‐pallidoluysian atrophy (DRPLA), and spinocerebellar ataxias 1, 2, 3, 6, 7 and 17 (SCAs). Although knockout and transgenic mouse experiments suggest that a toxic gain of function is central to ...
Elsdon Storey   +2 more
openaire   +3 more sources

Polyglutamine and Neurodegeneration: Structural Aspects

Protein & Peptide Letters, 2004
Polyglutamine (polyQ) diseases are inherited neurodegenerative disorders caused by proteins with expanded polyQ regions. Although the pathological mechanisms of these diseases have not yet been elucidated, the processes of protein misfolding and aggregation seem to be a direct cause of neurodegeneration.
openaire   +3 more sources

Death by Polyglutamine Tracts

Science Signaling, 2012
A polyglutamine repeat–containing protein functions in developmentally programmed, nonapoptotic cell death in the nematode.
openaire   +2 more sources

Gene Therapies for Polyglutamine Diseases

2018
Polyglutamine diseases are hereditary degenerative disorders of the nervous system that have remained, to this date, untreatable. Promisingly, investigation into their molecular etiology and the development of increasingly perfected tools have contributed to the design of novel strategies with therapeutic potential.
Carlos A. Matos   +8 more
openaire   +3 more sources

Faculty Opinions recommendation of CAG repeat not polyglutamine length determines timing of huntington's disease onset.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature, 2019
S. Humbert
semanticscholar   +1 more source

Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the α1A-voltage-dependent calcium channel

Nature Genetics, 1997
O. Zhuchenko   +9 more
semanticscholar   +1 more source

The CAG-polyglutamine repeat diseases: a clinical, molecular, genetic, and pathophysiologic nosology.

Handbook of Clinical Neurology, 2018
Colleen A. Stoyas, A. L. La Spada
semanticscholar   +1 more source

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

Nature, 2001
J. Steffan   +14 more
semanticscholar   +1 more source

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