Glutaric Aciduria I

History: Glutaric Aciduria Type I (GA I) was first described by Distinguished Professor Stephen Goodman in 1975. The Goodman laboratory, including Dr. Woontner, focused on GA I for over forty years. We are honored to continue this work at the University of Colorado.

Research: We are focused on the role of lysine metabolism, specifically the metabolites glutaric acid and 3-hydroxy-glutaric acid, in the development of striatal necrosis. Current treatments include reducing oral lysine intake and limiting catabolism with the goal of reducing the accumulation of 3-hydroxy-glutraic acid. Using zebrafish as a principal model, we aim to investigate the role of metabolism in disease pathophysiology and evaluate novel treatments for this neurologic disorder.