Formation and toxicity of soluble polyglutamine oligomers in living cells.

ABSTRACT:

Background

Aggregation and cytotoxicity of mutant proteins containing an expanded number of polyglutamine (polyQ) repeats is a hallmark of several diseases, including Huntington's disease (HD). Within cells, mutant Huntingtin (mHtt) and other polyglutamine expansion mutant proteins exist as monomers, soluble oligomers, and insoluble inclusion bodies (IBs). Determining which of these forms constitute a toxic species has proven difficult. Recent studies support a role for IBs as a cellular coping mechanism to sequester levels of potentially toxic soluble monomeric and oligomeric species of mHtt.

Methodology/principal findings

When fused to a fluorescent reporter (GFP) and expressed in cells, the soluble monomeric and oligomeric polyglutamine species are visually indistinguisha

SUBMITTER: Lajoie P 

PROVIDER: S-EPMC3011017 | biostudies-literature | 2010 Dec

REPOSITORIES: biostudies-literature