N-acetyl-L-cysteine prevents stress-induced desmin aggregation in cellular models of desminopathy.

Mutations within the human desmin gene are responsible for a subcategory of myofibrillar myopathies called desminopathies. However, a single inherited mutation can produce different phenotypes within a family, suggesting that environmental factors influence disease states. Although several mouse mod...

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Bibliographic Details
Main Authors: Bertrand-David Segard, Florence Delort, Virginie Bailleux, Stéphanie Simon, Emilie Leccia, Blandine Gausseres, Fatma Briki, Patrick Vicart, Sabrina Batonnet-Pichon
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2013-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3788106?pdf=render