Table of Contents:
  • GM2 gangliosidosis: the disease and the associated system of beta-hexosaminidase isozymes
  • Structure-function relationships
  • What have naturally occurring mutations told us?
  • Why is dimerization necessary for activity if both subunits have active sites?
  • What is the basis for the substrate-specificity differences between the active sites of the two subunits?
  • Why can only heterodimeric hexosaminidase A hydrolyze GM2 ganglioside?