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Aberrant Caspase Activation in Laminin-α2-Deficient Human Myogenic Cells is Mediated by p53 and Sirtuin Activity

BACKGROUND: Mutations in the LAMA2 gene encoding laminin-α2 cause congenital muscular dystrophy Type 1A (MDC1A), a severe recessive disease with no effective treatment. Previous studies have shown that aberrant activation of caspases and cell death through a pathway regulated by BAX and KU70 is a si...

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Detalles Bibliográficos
Autores principales: Yoon, Soonsang, Beermann, Mary Lou, Yu, Bryant, Shao, Di, Bachschmid, Markus, Miller, Jeffrey Boone
Formato: Online Artículo Texto
Lenguaje:English
Publicado: IOS Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5836413/
https://www.ncbi.nlm.nih.gov/pubmed/29278895
http://dx.doi.org/10.3233/JND-170262