Modulating the endoplasmic reticulum stress response attenuates neurodegeneration in a Caenorhabditis elegans model of spinal muscular atrophy

Spinal muscular atrophy (SMA) is a devastating autosomal recessive neuromuscular disease resulting in muscle atrophy and neurodegeneration, and is the leading genetic cause of infant death. SMA arises when there are homozygous deletion mutations in the human SMN1 gene, leading to a decrease in corre...

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Bibliographic Details
Main Authors: James J. Doyle, Celine Vrancx, Claudia Maios, Audrey Labarre, Shunmoogum A. Patten, J. Alex Parker
Format: Article
Language:English
Published: The Company of Biologists 2020-12-01
Series:Disease Models & Mechanisms
Subjects:
Online Access:http://dmm.biologists.org/content/13/12/dmm041350