GM2A : ganglioside GM2 activator
Description
The GM2A (ganglioside GM2 activator) is a protein-coding gene located on chromosome 5.
The GM2A gene provides instructions for making a protein called the ganglioside GM2 activator. This protein is necessary for the normal function of an enzyme called beta-hexosaminidase A. Beta-hexosaminidase A and the ganglioside GM2 activator protein work together in lysosomes, which are compartments in the cell that digest and recycle different types of molecules. Within lysosomes, the activator protein binds to a fatty substance called GM2 ganglioside and presents it to beta-hexosaminidase A to be broken down.
GM2A binds to gangliosides and stimulates the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. It extracts single GM2 molecules from membranes and presents them in soluble form to beta-hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3. GM2A also exhibits some calcium-independent phospholipase activity. GM2A has cholesterol transfer activity.
GM2A is also known as GM2-AP, GM2AP, SAP-3.
