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In Vivo and Ex Vivo Evaluation of L-Type Calcium Channel Blockers on Acid β-Glucosidase in Gaucher Disease Mouse Models

Gaucher disease is a lysosomal storage disease caused by mutations in acid β-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, t...

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Detalles Bibliográficos
Autores principales: Sun, Ying, Liou, Benjamin, Quinn, Brian, Ran, Huimin, Xu, You-Hai, Grabowski, Gregory A.
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2009
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2753664/
https://www.ncbi.nlm.nih.gov/pubmed/19809509
http://dx.doi.org/10.1371/journal.pone.0007320