pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

Expansion of CAG trinucleotide repeat within the coding region of several genes results in the production of proteins with expanded polyglutamine (PolyQ) stretch. The expression of these pathogenic proteins leads to PolyQ diseases, such as Huntington's disease or several types of spinocerebella...

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Veröffentlicht in:	Journal of neurochemistry 2009-09, Vol.110 (6), p.1737-1765
Hauptverfasser:	Bauer, Peter O, Nukina, Nobuyuki
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult and adolescent clinical studies Animals Biochemistry Biological and medical sciences CAG repeat Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Disease Models, Animal Genetic disorders Humans huntingtin Huntington's disease Medical sciences Mutation Mutation - genetics Nerve Tissue Proteins - genetics Neurodegenerative Diseases - classification Neurodegenerative Diseases - genetics Neurodegenerative Diseases - therapy Neurological disorders Neurology Neurons Organic mental disorders. Neuropsychology pathogenesis Pathology Peptides - genetics polyglutamine Protein Folding Proteins Psychology. Psychoanalysis. Psychiatry Psychopathology. Psychiatry therapeutics therapy Trinucleotide Repeat Expansion - genetics
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