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Abstract #0471

Axonal Injury and Myelin Loss in Glutaric Acidemia Type I (GA-1) Mouse Model of Diet Induced Encephalopathy

Jelena Lazovic1, William J. Zinnanti2, Russell E. Jacobs1

1Biology, California Institute of Technology, Pasadena, CA, United States; 2Pediatrics, Children's hospital at SUNY Downstate, Brooklyn, NY, United States

In recent years white matter abnormalities, including leukoencephalopathy, are being increasingly recognized in patients suffering from glutaric acidemia type I (GA-1). The mechanism leading to leukoencephalopathy remains unknown, as well as the extent of myelin degradation. In this work we use a mouse model of GA-1 and combination of MRI, histology and behavioral testing to establish the basis for abnormal appearance of white matter in this disorder. Presented data suggest myelin degradation to be secondary to axonal loss in GA-1. Behavioral data implicate damaged neuronal populations to be involved in sensory-motor integration.