Danon Disease-Associated LAMP-2 Deficiency Drives Metabolic Signature Indicative of Mitochondrial Aging and Fibrosis in Cardiac Tissue and hiPSC-Derived Cardiomyocytes

Danon disease is a severe X-linked disorder caused by deficiency of the lysosome-associated membrane protein-2 (LAMP-2). Clinical manifestations are phenotypically diverse and consist of hypertrophic and dilated cardiomyopathies, skeletal myopathy, retinopathy, and intellectual dysfunction. Here, we...

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Detalles Bibliográficos
Autores principales: Del Favero, Giorgia, Bonifacio, Alois, Rowland, Teisha J., Gao, Shanshan, Song, Kunhua, Sergo, Valter, Adler, Eric D., Mestroni, Luisa, Sbaizero, Orfeo, Taylor, Matthew R. G.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7465084/
https://www.ncbi.nlm.nih.gov/pubmed/32751926
http://dx.doi.org/10.3390/jcm9082457

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7465084/
https://www.ncbi.nlm.nih.gov/pubmed/32751926
http://dx.doi.org/10.3390/jcm9082457

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