Mitochondrial Dysfunction and Calcium Dysregulation in Leigh Syndrome Induced Pluripotent Stem Cell Derived Neurons

Mitochondrial Dysfunction and Calcium Dysregulation in Leigh Syndrome Induced Pluripotent Stem Cell Derived Neurons

Leigh syndrome (LS) is the most frequent infantile mitochondrial disorder (MD) and is characterized by neurodegeneration and astrogliosis in the basal ganglia or the brain stem. At present, there is no cure or treatment for this disease, partly due to scarcity of LS models. Current models generally...

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Bibliographic Details
Main Authors: Teresa Galera-Monge, Francisco Zurita-Díaz, Isaac Canals, Marita Grønning Hansen, Laura Rufián-Vázquez, Johannes K. Ehinger, Eskil Elmér, Miguel A. Martin, Rafael Garesse, Henrik Ahlenius, M. Esther Gallardo
Format: Article
Language:English
Published: MDPI AG 2020-04-01
Series:International Journal of Molecular Sciences
Subjects:
Leigh syndrome
mitochondrial disorder
iPSC
NSC
neuron
disease modeling
Online Access:https://www.mdpi.com/1422-0067/21/9/3191

Internet

https://www.mdpi.com/1422-0067/21/9/3191

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