Our work is to understand the regulation and function of the heat shock transcriptional response (HSR) as a proteome quality control (QC) mechanism. The rationale and import of this work include: (1) the acknowledged role of protein QC in stress resistance and longevity, and (2) mutations and/or dysfunction of protein QC contribute to pathogenesis in protein conformation diseases, notably age-related neurodegenerative diseases (ND) such as Alzheimer’s, Parkinson’s, Lou Gehrig’s, Huntington’s, and Prion (CJD) diseases. Our recent study on the role of heat shock and HSP chaperones on mHtt yielded important insights on mHtt structuring, aggregation, and disease pathogenesis. This observation likely is relevant to our understanding of the disease mechanisms in other ND. We are confident of the import of this work and the impact in charting future therapeutics development.
