GM2 activator protein

Abstract

GM2 activator protein is a protein cofactor which has been found to be intimately related to the catabolism of GM2-ganglioside. The physiological importance of GM2 activator protein has been shown by the fact that a deficiency of this activator protein results in a massive cerebral accumulation of GM2. However, the mechanism of action and the biological function of this activator protein are still not well understood. The purpose of this essay is to briefly recollect the personal accounts of our involvement with GM2 activator protein and also to discuss four unsettled questions pertaining to the specificity and the mechanism of action of this intriguing protein cofactor.