In vivo and ex vivo evaluation of L-type calcium channel blockers on acid beta-glucosidase in Gaucher disease mouse models.

In vivo and ex vivo evaluation of L-type calcium channel blockers on acid beta-glucosidase in Gaucher disease mouse models.

Gaucher disease is a lysosomal storage disease caused by mutations in acid beta-glucosidase (GCase) leading to defective hydrolysis and accumulation of its substrates. Two L-type calcium channel (LTCC) blockers-verapamil and diltiazem-have been reported to modulate endoplasmic reticulum (ER) folding...

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Bibliographic Details
Main Authors: Ying Sun, Benjamin Liou, Brian Quinn, Huimin Ran, You-Hai Xu, Gregory A Grabowski
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2009-10-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC2753664?pdf=render

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