- Author:
Sangjune KIM
1
;
Kyong Tai KIM
Author Information
- Publication Type:Review
- Keywords: protein aggregation; Molecular chaperone; Huntington; anti-aggregation drug
- MeSH: Huntington Disease*; Models, Animal; Molecular Chaperones; Neurodegenerative Diseases
- From:Experimental Neurobiology 2014;23(1):36-44
- CountryRepublic of Korea
- Language:English
- Abstract: Huntington's disease (HD) is a late-onset and progressive neurodegenerative disorder that is caused by aggregation of mutant huntingtin protein which contains expanded-polyglutamine. The molecular chaperones modulate the aggregation in early stage and known for the most potent protector of neurodegeneration in animal models of HD. Over the past decades, a number of studies have demonstrated molecular chaperones alleviate the pathogenic symptoms by polyQ-mediated toxicity. Moreover, chaperone-inducible drugs and anti-aggregation drugs have beneficial effects on symptoms of disease. Here, we focus on the function of molecular chaperone in animal models of HD, and review the recent therapeutic approaches to modulate expression and turn-over of molecular chaperone and to develop anti-aggregation drugs.