The Johnson lab seeks to uncover novel triggers of neuronal death in two neurodegenerative diseases, amyotrophic lateral Sclerosis (ALS) and frontotemporal dementia (FTD). While ALS and FTD differ in their clinical presentation, they are both characterized by the misfolding and aggregation of proteins in and around neurons, termed protein aggregates. Proteins are macromolecules that perform essential functions for the cell including providing structure, acting as catalysts, and signaling molecules. Different cells express different proteins at different timepoints. Accumulation of certain proteins at counterproductive times can lead to protein aggregation, a progressive process whereby proteins misfold and group together in or around the cell. Current research aims to identify (1) whether changes in protein expression during embryonic development influence neurodegeneration risk (2) novel triggers of protein aggregation and determine (2) the role protein aggregation plays in cellular death.
