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Polyglutamine conformation in GST-polyglutamine fusion proteins [PDF]
Annalisa Pastore+4 more
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Therapeutic opportunities in polyglutamine disease
Nature Medicine, 2001Polyglutamine 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
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Role of proteolysis in polyglutamine disorders
Journal of Neuroscience Research, 2003AbstractTo 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
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Polyglutamine and Neurodegeneration: Structural Aspects
Protein & Peptide Letters, 2004Polyglutamine (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.
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Science Signaling, 2012
A polyglutamine repeat–containing protein functions in developmentally programmed, nonapoptotic cell death in the nematode.
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A polyglutamine repeat–containing protein functions in developmentally programmed, nonapoptotic cell death in the nematode.
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Gene Therapies for Polyglutamine Diseases
2018Polyglutamine 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
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Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature, 2019
S. Humbert
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S. Humbert
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Handbook of Clinical Neurology, 2018
Colleen A. Stoyas, A. L. La Spada
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Colleen A. Stoyas, A. L. La Spada
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Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila
Nature, 2001J. Steffan+14 more
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