Cortical transcriptome reveals widespread inflammation in brain of Gaa-/- mice. Cortical transcriptome reveals widespread inflammation in brain of Gaa-/- mice

Glycogen storage disease type II (Pompe's disease) is a lysosomal storage disorder which is associated with intralysosomal accumulation of glycogen impais muscle and nerve function. Mice that lack the lysosomal enzyme, acid alpha glucosidase model the human Pompe's disease. To understand signaling mechanisms that could potentially drive the progression of the disease, we employed RNA-Seq to unbiasedly characterize transcriptional changes in the cortex of Gaa-/- mice. We studied Gaa-/- mice and their wild type littermates at 12 months of age and found a robust induction of inflammatory genes in the cortex of Gaa-/- mice. Overall design: WT and Gaa KO mice at 12 months of age