Project Summary
This project aims to understand the role of the lysosmal enzyme Hexb for proper macrophage homeostasis in the brain. To date the mechanisms by which the lack of Hexb induces neuroinflammatory degeneration of the CNS is poorly understood. The project will therefore A) explore the expression and regulation of Hexb in several myeloid cell compartments, B) investigate the molecular mechanisms by which mutant microglia contribute to disease, C) determine the mechanisms of GM2 ganglioside accumulation in tissue stromal cells and finally, D) characterize the molecular signature in human Sandhoff disease specimen.
