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

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, electrophy...

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Detalles Bibliográficos
Autores principales: Yoon, Jin‐Young, Daneshgar, Nastaran, Chu, Yi, Chen, Biyi, Hefti, Marco, Vikram, Ajit, Irani, Kaikobad, Song, Long‐Sheng, Brenner, Charles, Abel, E. Dale, London, Barry, Dai, Dao‐Fu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Research Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9309541/
https://www.ncbi.nlm.nih.gov/pubmed/35872650
http://dx.doi.org/10.1002/ctm2.954

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