EXPANDING THE TRUHD CELL LIBRARY OF HUNTINGTON’S DISEASE

Abstract

Huntington’s disease (HD) is a late-onset neurodegenerative disorder caused by the expansion of the CAG repeat in the HTT allele. This forms an expanded huntingtin protein which disrupts various cellular processes including DNA damage repair pathways. Many models have been developed to study HD. This includes the TruHD cells which are immortal patient-derived fibroblasts that retain characteristics of their original primary culture. However, the current TruHD cell library is limited. In this project, we expanded the library of TruHD cells, characterized their basal growth rate and huntingtin levels, identified a standard set of cells for future experiments, and amended the TruHD name to better guide future investigations. Furthermore, we developed and optimized qPCR as a tool for the TruHD cells. N6-furfuryladenine was also assessed and determined to not modulate HTT mRNA levels and not induce apoptosis in control cells.