Article
Progranulin Recruits HSP70 to β-Glucocerebrosidase and Is Therapeutic Against Gaucher Disease.
EBioMedicine - 1 Nov 2016
Jian Jinlong, Tian Qing-Yun, Hettinghouse Aubryanna, Zhao Shuai, Liu Helen, Wei Jianlu, Grunig Gabriele, Zhang Wujuan, Setchell Kenneth D R, Sun Ying, Overkleeft Herman S, Chan Gerald L, Liu Chuan-Ju
Abstract excerpt
Gaucher disease (GD), the most common lysosomal storage disease, is caused by mutations in GBA1 encoding of β-glucocerebrosidase (GCase). Recently it was reported that progranulin (PGRN) insufficiency and deficiency associated with GD in human and mice, respectively. However the underlying mechanisms remain unknown. Here we report that PGRN binds directly to GCase and its deficiency results in aggregation of...
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