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. The gene discussed is SNCA; the disease is Sandhoff disease.