Molecular and Cellular Neuroscience Commons^™

The Leucine-Rich Domain Of Rgnef: A Modifier Of Tdp-43 Toxicity In Drosophila Melanogaster., Benjamin Martin Withers

Electronic Thesis and Dissertation Repository

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the progressive degeneration of motor neurons. Rho Guanine Nucleotide Exchange factor (RGNEF) like other RNA-binding proteins, has been observed to form inclusions in the spinal cord motor neurons of both sporadic and familial cases of ALS. RGNEF has been determined to be a pro-survival factor under stress conditions. When comparing expression of different constructs of RGNEF in HEK293T cells, a Leucine-rich domain containing fragment of RGNEF (L-Rich) was found to form aggregates under metabolic stress that co-aggregated with TDP-43, another ALS-linked RNA-binding protein.

In this thesis, I used both …