Characterization of the affinity of the G(M2) activator protein for glycolipids by a fluorescence dequenching assay

Biochim Biophys Acta. 1997 May 23;1339(2):192-202. doi: 10.1016/s0167-4838(97)00002-2.

Abstract

The G(M2) activator protein is a substrate specific cofactor for degradation of G(M2) ganglioside by lysosomal beta-hexosaminidase A. Mutations in the gene encoding the activator result in the AB-variant form of G(M2) gangliosidosis. The activator protein contains at least three functional elements; a hydrophobic binding pocket, an oligosaccharide binding site(s), and an area that interacts with hexosaminidase A. In this report a fluorescence dequenching assay specific for only the hydrophobic binding pocket is evaluated and optimized. It is shown that various glycolipids inhibit the transport between liposomes of a self-quenching fluorescent lipid probe, octadecylrhodamine, by the activator protein. The level of inhibition produced by each glycolipid is then used to characterize the oligosaccharide-binding specificity of the activator. The fluorescence dequenching assay is also used to evaluate the functionality of a truncated form of the activator protein. Our results indicate that this simple assay can be used to determine structure-function relationships within the normal or mutant forms of the activator. The data suggest that the C-terminus of the activator is required to produce a functional hydrophobic binding pocket.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Binding Sites
  • Carbohydrate Sequence
  • Cloning, Molecular
  • Escherichia coli / genetics
  • Fluorescence
  • G(M2) Activator Protein
  • G(M2) Ganglioside / metabolism*
  • Glycolipids / metabolism*
  • Hexosaminidase A
  • Molecular Sequence Data
  • Oligosaccharides / chemistry
  • Polymerase Chain Reaction
  • Proteins / chemistry
  • Proteins / genetics
  • Proteins / metabolism*
  • Structure-Activity Relationship
  • beta-N-Acetylhexosaminidases / metabolism

Substances

  • G(M2) Activator Protein
  • Glycolipids
  • Oligosaccharides
  • Proteins
  • G(M2) Ganglioside
  • Hexosaminidase A
  • beta-N-Acetylhexosaminidases