NAG-thiazoline, NGT, acts as mechanism based inhibitor of hexosaminidase A. In patients with Tay–Sachs disease (misfolded hexosaminidase A), NGT acts as a molecular chaperone by binding in the active site of hexosaminidase A which helps create a properly folded hexosaminidase A.
NAG-thiazoline, NGT, is used to treat Tay–Sachs disease by inhibiting the activity of hexosaminidase A and assisting in its proper folding.