Automated description from the Alliance of Genome Resources (Release 7.0.0)
Enables beta-N-acetylhexosaminidase activity and enzyme activator activity. Acts upstream of or within several processes, including ganglioside catabolic process; lipid storage; and neuromuscular process controlling balance. Located in mitochondrion. Is expressed in several structures, including back skin; central nervous system; genitourinary system; hemolymphoid system; and retina. Used to study GM2 gangliosidosis, AB variant. Human ortholog(s) of this gene implicated in GM2 gangliosidosis and GM2 gangliosidosis, AB variant. Orthologous to human GM2A (ganglioside GM2 activator).