<unk>  is a rare, autosomal recessive metabolic disorder that causes progressive destruction of nerve cells in the brain and spinal cord. It has a similar pathology to Sandhoff disease and Tay–Sachs disease. The three diseases are classified together as the GM2<unk> , because each disease represents a distinct molecular point of failure in the activation of the same enzyme, beta-hexosaminidase.<unk><unk>  is caused by a failure in the gene that makes an enzyme cofactor for beta-hexosaminidase, called the GM2 activator.
GM2-gangliosidosis, AB variant
GM1 gangliosidoses
GM2 gangliosidoses
Adult polyglucosan body disease
Mucopolysaccharidosis type I
Glycogen storage disease type 0
Mucolipidosis type IV
Griscelli syndrome type 2