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

Metabolic Maturation Exaggerates Abnormal Calcium Handling in a <i>Lamp2</i> 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 (<i>LAMP2</i>), which lead to impaired autophagy, glycogen accumulation, and cardiac hypertrophy. However, it is not well understood why a large portion of DD patients develop arrh...

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Bibliographic Details
Main Authors: Robert J. Barndt, Qing Liu, Ying Tang, Michael P. Haugh, Jeffery Cui, Stephen Y. Chan, Haodi Wu
Format: Article
Language:English
Published: MDPI AG 2022-12-01
Series:Biomolecules
Subjects:
Danon disease
LAMP2
iPSC-derived cardiomyocyte
metabolic maturation
arrhythmia
Online Access:https://www.mdpi.com/2218-273X/13/1/69

Internet

https://www.mdpi.com/2218-273X/13/1/69

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