Replication Data for: Granulins rescue inflammation, lysosome dysfunction, and neuropathology in a mouse model of progranulin deficiency

Progranulin (PGRN) deficiency is linked to neurodegenerative diseases including frontotemporal dementia (FTD), Alzheimer’s, and Parkinson’s disease. Proper PGRN levels are critical for brain health, however the function of PGRN is unclear. PGRN is composed of 7.5 repeat domains, called granulins, and is processed into granulins inside the lysosome. The beneficial effects of PGRN are known, but the role of granulins is unclear. In the linked publication we report our discovery that AAV-mediated expression of single granulins rescues disease pathology in Grn-/- (knockout) mice. Expression of human granulin-2/F, granulin-4/A, or PGRN in Grn-/- mouse brain ameliorated dysregulated lysosomal proteins and lipids, microgliosis, and lipofuscinosis. This data set includes the annotated mass spectrometry data of lipidomics and metabolomics analyses of the frontal cortex of GFP-Grn+/+, GFP-Grn-/-, hPGRN-Grn-/-, hGRN2-Grn-/-, and hGrn4-Grn-/- mice.