The Nitric Oxide Donor, S-Nitrosoglutathione, Rescues Peroxisome Number and Activity Defects in PEX1G843D Mild Zellweger Syndrome Fibroblasts

The Nitric Oxide Donor, S-Nitrosoglutathione, Rescues Peroxisome Number and Activity Defects in PEX1G843D Mild Zellweger Syndrome Fibroblasts

Peroxisome biogenesis disorders (PBDs) are a group of metabolic developmental diseases caused by mutations in one or more genes encoding peroxisomal proteins. Zellweger syndrome spectrum (PBD-ZSS) results from metabolic dysfunction caused by damaged or non-functional peroxisomes and manifests as a m...

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Bibliographic Details
Main Authors: Yidi Liu, Ceileigh M. Weaver, Yarina Sen, Gary Eitzen, Andrew J. Simmonds, Lilliana Linchieh, Olivier Lurette, Etienne Hebert-Chatelain, Richard A. Rachubinski, Francesca Di Cara
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-08-01
Series:Frontiers in Cell and Developmental Biology
Subjects:
peroxisome
PEX1G843D mutation
Zellweger spectrum disorders
S-nitrosoglutathione
high-throughput screen
drug library
Online Access:https://www.frontiersin.org/articles/10.3389/fcell.2021.714710/full

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