Formation of a ternary complex between GM2 activator protein, GM2 ganglioside and hexosaminidase A
F Yadao, P Hechtman, F Kaplan - Biochimica et Biophysica Acta (BBA) …, 1997 - Elsevier
The GM2 activator is a 17 kDa protein required for the hydrolysis of GM2 ganglioside by the
lysosomal enzyme hexosaminidase A (HexA). The activator behaves as a substrate binding
protein, solubilizing GM2 ganglioside monomers from micelles (in vitro) or membranes (in
vivo). However, the activator also shows a high order of specificity for activation of lysosomal
hydrolases and has been predicted to form a ternary complex with the heterodimeric
enzyme (αβ) HexA and GM2 ganglioside. We demonstrated a transient interaction between …
lysosomal enzyme hexosaminidase A (HexA). The activator behaves as a substrate binding
protein, solubilizing GM2 ganglioside monomers from micelles (in vitro) or membranes (in
vivo). However, the activator also shows a high order of specificity for activation of lysosomal
hydrolases and has been predicted to form a ternary complex with the heterodimeric
enzyme (αβ) HexA and GM2 ganglioside. We demonstrated a transient interaction between …