Jelena Lazovic1, William J. Zinnanti2, Xiaowei Zhang3, Russell Jacobs3
1Radiology, University of California, Los Angeles, Los Angeles, CA, United States; 2Neurology, Stanford, Palo Alto, CA, United States; 3California Institute of Technology
The aim of this work was to investigate whether local increases in glutaric acid, a metabolite that accumulates in glutaric academia type 1 (GA-1), can lead to retrograde neuronal injury. In this metabolic disorder, the striatum appears to be highly vulnerable brain region, without apparent high glutaric acid content. We hypothesized that increase in glutaric acid levels in areas outside the striatum can cause distal striatal injury. The addition of glutaric acid to the thalamus, induced neuronal injury in the substantia nigra, while cortical injection of glutaric acid resulted in localized striatal injury.