Articles

  • the Korean Society for Brain and Neural Sciences

Article

Review Article

Exp Neurobiol 2014; 23(1): 36-44

Published online March 30, 2014

https://doi.org/10.5607/en.2014.23.1.36

© The Korean Society for Brain and Neural Sciences

Therapeutic Approaches for Inhibition of Protein Aggregation in Huntington’s Disease

Sangjune Kim1 and Kyong-Tai Kim1,2*

1Department of Life Sciences, 2Division of Integrative Biosciences and Biotechnology, Pohang University of Science and Technology, Pohang 790-784, Korea

Correspondence to: *To whom correspondence should be addressed.
TEL: 82-54-279-2297, FAX: 82-54-279-2297
e-mail: ktk@postech.ac.kr

Received: December 9, 2013; Revised: December 19, 2013; Accepted: December 20, 2013

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.

Keywords: protein aggregation, Molecular chaperone, Huntington, anti-aggregation drug