Article
An evolutionary and structure-based docking model for glucocerebrosidase-saposin C and glucocerebrosidase-substrate interactions - relevance for Gaucher disease.
Proteins - 15 Feb 2008
Atrian Sílvia, López-Viñas Eduardo, Gómez-Puertas Paulino, Chabás Amparo, Vilageliu Lluïsa, Grinberg Daniel
Abstract excerpt
Gaucher disease, the most prevalent lysosomal storage disorder, is principally caused by malfunction of the lysosomal enzyme glucocerebrosidase (GBA), a 497-amino acid membrane glycoprotein that catalyzes the hydrolysis of glucosylceramide to ceramide and glucose in the presence of an essential 84-residue activator peptide named saposin C (SapC). Knowledge of the GBA structure, a typical (beta/alpha)(8) TIM...
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