Metabolic rescue ameliorates mitochondrial encephalo‐cardiomyopathy in murine and human iPSC models of Leigh syndrome

Metabolic rescue ameliorates mitochondrial encephalo‐cardiomyopathy in murine and human iPSC models of Leigh syndrome

Abstract Background Mice with deletion of complex I subunit Ndufs4 develop mitochondrial encephalomyopathy resembling Leigh syndrome (LS). The metabolic derangement and underlying mechanisms of cardio‐encephalomyopathy in LS remains incompletely understood. Methods We performed echocardiography, ele...

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Bibliographic Details
Main Authors: Jin‐Young Yoon, Nastaran Daneshgar, Yi Chu, Biyi Chen, Marco Hefti, Ajit Vikram, Kaikobad Irani, Long‐Sheng Song, Charles Brenner, E. Dale Abel, Barry London, Dao‐Fu Dai
Format: Article
Language:English
Published: Wiley 2022-07-01
Series:Clinical and Translational Medicine
Subjects:
Cardio‐encephalomyopathy
Leigh syndrome
mitochondria
Ndufs
nicotinamide riboside
Online Access:https://doi.org/10.1002/ctm2.954

Internet

https://doi.org/10.1002/ctm2.954

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