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Metabolic Maturation Exaggerates Abnormal Calcium Handling in a Lamp2 Knockout Human Pluripotent Stem Cell-Derived Cardiomyocyte Model of Danon Disease

Danon disease (DD) is caused by mutations of the gene encoding lysosomal-associated membrane protein type 2 (LAMP2), which lead to impaired autophagy, glycogen accumulation, and cardiac hypertrophy. However, it is not well understood why a large portion of DD patients develop arrhythmia and sudden c...

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Detalles Bibliográficos
Autores principales: Barndt, Robert J., Liu, Qing, Tang, Ying, Haugh, Michael P., Cui, Jeffery, Chan, Stephen Y., Wu, Haodi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9855424/
https://www.ncbi.nlm.nih.gov/pubmed/36671453
http://dx.doi.org/10.3390/biom13010069