Patients with Gaucher disease display systemic oxidative stress dependent on therapy status

Gaucher disease is an autosomal recessive metabolic disorder caused by mutations in GBA1, which encodes for the lysosomal hydrolase enzyme, β-glucocerebrosidase. The resulting misfolded protein can trigger endoplasmic reticulum stress and an unfolded protein response within the affected cells. The e...

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Bibliographic Details
Main Authors: Reena V. Kartha, Marcia R. Terluk, Roland Brown, Abigail Travis, Usha R. Mishra, Kyle Rudser, Heather Lau, Jeanine R. Jarnes, James C. Cloyd, Neal J. Weinreb
Format: Article
Language:English
Published: Elsevier 2020-12-01
Series:Molecular Genetics and Metabolism Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2214426920301130