Biochemical and Mechanical Dysfunction in a Mouse Model of Desmin-Related Myopathy

An R120G mutation in αB-crystallin (CryAB(R120G)) causes desmin-related myopathy (DRM). In mice with cardiomyocyte-specific expression of the mutation, CryAB(R120G)-mediated DRM is characterized by CryAB and desmin accumulations within cardiac muscle, mitochondrial deficiencies, activation of apopto...

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Autors principals: Maloyan, Alina, Osinska, Hanna, Lammerding, Jan, Lee, Richard T., Cingolani, Oscar H., Kass, David A., Lorenz, John N., Robbins, Jeffrey
Format: Artigo
Idioma:Inglês
Publicat: 2009
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Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4153728/
https://ncbi.nlm.nih.gov/pubmed/19299643
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1161/CIRCRESAHA.108.193516
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