The GM2-activator protein (GM2AP) is an essential
cofactor for the degradation of ganglioside GM2 by lyso-somal b-hexosaminidase A. It mediates the interaction
between the water-soluble exohydrolase and its membrane-bound substrate at the lipid–water interphase. Inherited
defects in the gene encoding this glycoprotein result ina fatal
neurological storage disorder, the AB variant of GM2-gangliosidosis. To elucidate the mode of action of this gly-coprotein cofactor, we synthesized the two photoaffinity
-TPD-GM2 and [
The domain of catalytic hydrogenation continues to grow fast, reflecting the wide range of chemical applications that can be enhanced by the easy use of molecular hydrogen. The advances in characterization techniques and their application have improved our understanding of the catalytic processes and mechanisms occurring in both homogeneous and heterogeneous catalysis.
a-Conotoxins are small peptides from cone snail venoms that function as
nicotinic acetylcholine receptor (nAChR)-competitive antagonists differenti-ating between nAChR subtypes. Current understanding about the mechan-ism of these selective interactions is based largely on mutational analyses,
which identify amino acids in the toxin and nAChR that determine the
energetics of ligand binding.