Abstract:
: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 spinocerebellar ataxias. This family of neurodegenerative disorders is characterized by constant progression of the symptoms and molecularly, by the accumulation of mutant proteins inside neurons causing their dysfunction and eventually death. So far, no effective therapy actually preventing the physical and/or mental decline has been developed. Experimental therapeutic strategies either target the levels or processing of mutant proteins in an attempt to prevent cellular deterioration, or they are aimed at the downstream pathologic effects to reverse or ameliorate the caused damages. Certain pathomechanistic aspects of PolyQ disorders are discussed here. Relevance of disease models and recent knowledge of therapeutic possibilities is reviewed and updated.
journal_name
J Neurochemjournal_title
Journal of neurochemistryauthors
Bauer PO,Nukina Ndoi
10.1111/j.1471-4159.2009.06302.xsubject
Has Abstractpub_date
2009-09-01 00:00:00pages
1737-65issue
6eissn
0022-3042issn
1471-4159pii
JNC6302journal_volume
110pub_type
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