Activation and Purification of ß-Glucocerebrosidase by Exploiting its Transporter LIMP-2 - Implications for Novel Treatment Strategies in Gaucher's and Parkinson's Disease.
Authors:Dobert JP,Bub S,Mächtel R,Januliene D,Steger L,Regensburger M,Wilfling S,Chen JX,Dejung M,Plötz S,Hehr U,Moeller A,Arnold P,Zunke F
Journal:Advanced science (Weinheim, Baden-Wurttemberg, Germany)
PubMed ID:38666485
Genetic variants of GBA1 can cause the lysosomal storage disorder Gaucher disease and are among the highest genetic risk factors for Parkinson's disease (PD). GBA1 encodes the lysosomal enzyme beta-glucocerebrosidase (GCase), which orchestrates the degradation of glucosylceramide (GluCer) in the lysosome. Recent studies have shown that GluCer accelerates α-synuclein aggregation, ... More