ANALYSIS OF INTRACELLULAR PEROXIN PROTEIN PRODUCTION IN A TISSUE CULTURE MODEL
David Cortez, Edwin Paz.
University of California, San Diego, La Jolla, CA.
Peroxisome biogenesis disorders (PBD) share many features associated with neurodegenerative diseases including altered intracellular metabolism. PBDs are caused by peroxin (PEX) mutations resulting in peroxisome defects. Peroxisomes are organelles that regulate complex pathways including breakdown of reactive oxygen species (ROS) and beta oxidation of very long chain fatty acids (VLCFA). Developmental defects of this organelle result in brain disorders such as Zellweger syndrome. We are currently attempting to determine the effects of compounds that may alter PEX protein production that may lead to effects on intracellular peroxisome levels. We hypothesize that peroxisomes are tractable targets in neurodegenerative diseases. Using protein assays such as western blot, we are charactering the effects of candidate compounds on PEX gene production. Studying these compounds and their associated mechanisms of action may lead to novel treatment modalities.