Tags: Disease.
GM2-gangliosidosis AB variant 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 gangliosidoses because each disease represents a distinct molecular point of failure in the activation of the same enzyme beta-hexosaminidase.