Neural stem cells

Direct selection of neuroprotective intrabodies for Huntington’s disease

Charles W. Bugg, Ali Khoshnan, Paul H. Patterson

Mutant huntingtin (mHtt) causes Huntington’s disease (HD), a progressive and fatal neurodegenerative disorder. Intracellular antibodies (intrabodies) directed to different epitopes within exon 1 of mHtt have a variety of effects on the toxic protein. They can increase its turnover (intrabodies to the multiple proline domain), exacerbate its aggregation and toxicity (intrabodies to the polyglutamine region), or alter its cellular localization (intrabodies to the N-terminus). We are attempting to isolate intrabodies to novel epitopes of mHtt and characterize the role that these epitopes play in the cellular function and toxicity of mHtt. To this end, we have selected a panel of 30 intrabodies by phage display and are in the process of determining to which epitopes they bind. After determination of the epitopes, the contribution of the epitopes to mHtt toxicity will be characterized.

Back to Main Menu

This page last updated August, 2008 by C. Patterson.