P-187 LYSINE AND GLUTARIC ACID AFFECTS CULTURED ASTROCYTES FROM THE KNOCKOUT MOUSE MODEL OF GLUTARIC ACIDEMIA TYPE I 2011
Background: The role of glial cells in glutaric acidemia I (GA-I) pathogenesis is mostly unknown. Objectives: We evaluated the effects of pathophysiological concentrations of glutaric acid (GA) and lysine on glial cells from the glutaryl-CoA dehydrogenase (GCDH) knockout (KO) mouse model. Methods: Functional and morphological parameters were...