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Polyglutamine Diseases and Molecular Chaperones
IUBMB Life, 2003AbstractThe polyglutamine diseases, a group of diseases currently thought to consist of nine inherited neurodegenerative diseases, are caused by the expansion of unstable CAG trinucleotide repeats that code for polyglutamine tracts in the responsible genes.
Yoko, Kimura, Akira, Kakizuka
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Polyglutamine protein aggregates are dynamic
Nature Cell Biology, 2002Protein aggregation and the formation of inclusion bodies are hallmarks of the cytopathology of neurodegenerative diseases, including Huntington's disease, Amyotropic lateral sclerosis, Parkinson's disease and Alzheimer's disease. The cellular toxicity associated with protein aggregates has been suggested to result from the sequestration of essential ...
Soojin, Kim +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.
R E, Hughes, J M, Olson
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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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Polyglutamine conformation in GST-polyglutamine fusion proteins
Biochemical Society Transactions, 2002L. Masino, A. Pastore
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Polyglutamine expansion neurodegenerative disease
Brain Research Bulletin, 2001Kennedy's disease was the first of eight neurodegenerative disorders found to be caused by expanded polyglutamine repeats. Each of these disorders is likely caused by a toxic gain of function in the disease gene product, often associated with inclusions of mutant protein in susceptible neurons.
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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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Polyglutamine Diseases: Where does Toxicity Come from? What is Toxicity? Where are We Going?
Journal of Molecular Cell Biology, 2010Osamu Onodera, Onodera Osamu
exaly