Gene editing improves endoplasmic reticulum-mitochondrial contacts and unfolded protein response in Friedreich’s ataxia iPSC-derived neurons

Gene editing improves endoplasmic reticulum-mitochondrial contacts and unfolded protein response in Friedreich’s ataxia iPSC-derived neurons

Friedreich ataxia (FRDA) is a multisystemic, autosomal recessive disorder caused by homozygous GAA expansion mutation in the first intron of frataxin (FXN) gene. FXN is a mitochondrial protein critical for iron-sulfur cluster biosynthesis and deficiency impairs mitochondrial electron transport chain...

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Bibliographic Details
Main Authors: Priyanka Mishra, Anusha Sivakumar, Avalon Johnson, Carla Pernaci, Anna S. Warden, Lilas Rony El-Hachem, Emily Hansen, Rafael A. Badell-Grau, Veenita Khare, Gabriela Ramirez, Sydney Gillette, Angelyn B. Solis, Peng Guo, Nicole Coufal, Stephanie Cherqui
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-02-01
Series:Frontiers in Pharmacology
Subjects:
Friedreich’s ataxia
induced pluripotent stem cells
neurons
gene editing
neuronal apoptosis
calcium homeostasis
Online Access:https://www.frontiersin.org/articles/10.3389/fphar.2024.1323491/full

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https://www.frontiersin.org/articles/10.3389/fphar.2024.1323491/full

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