Exp Neurobiol.  2014 Mar;23(1):36-44. 10.5607/en.2014.23.1.36.

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

• Kim S
• Kim KT

• KMID: 2009023
• DOI: http://doi.org/10.5607/en.2014.23.1.36

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.

Keyword

protein aggregation; Molecular chaperone; Huntington; anti-aggregation drug

MeSH Terms

Huntington Disease*
Models, Animal
Molecular Chaperones
Neurodegenerative Diseases
Molecular Chaperones