Fig. 6: The structural basis of substrate selectivity and the molecular difference underlying GSD III.
From: Molecular architecture and catalytic mechanism of human glycogen debranching enzyme
a The structural basis of substrate selectivity in hsGDE GT domain. The selectivity for proper number glycosyl units is achieved by the size and the specifical chemical environment of the binding pocket. Lines in green indicate the H-bond, and circles mark the hydrophobic interaction. b Mutations that underlie GSD III trigger the disease through diverse pathways, including but not limited to impairment of catalytic function (magenta), reduction in substrate affinity (blue), disruption of structural integrity (orange), and other pathways (green), thereby manifesting the molecular differences that underlie this condition. Disease-causing mutations are shown in sphere form with only backbone.
