Thymic Alterations in GM2 Gangliosidoses Model Mice

Sandhoff disease is a lysosomal storage disorder characterized by the absence of β-hexosaminidase and storage of GM2 ganglioside and related glycolipids. We have previously found that the progressive neurologic disease induced in Hexb？/？ mice, an animal model for Sandhoff disease, is associated with the production of pathogenic anti-glycolipid autoantibodies.