To assess the impact of loss of lysosomal hydrolase ß-hexosaminidase (HEX) activity and concurrent ganglioside GM2 accumulation on alpha-synuclein (aSYN) homeostasis, total aSYN content was measured in whole brain samples collected from HEXB-deficient Sandhoff disease (SD) mice. Here, HEXB is linked to Sandhoff disease.