Article
Sonlicromanol improves neuronal network dysfunction and transcriptome changes linked to m.3243A>G heteroplasmy in iPSC-derived neurons.
Stem cell reports - 14 Sept 2021
Klein Gunnewiek Teun M, Verboven Anouk H A, Pelgrim Iris, Hogeweg Mark, Schoenmaker Chantal, Renkema Herma, Beyrath Julien, Smeitink Jan, de Vries Bert B A, Hoen Peter-Bram A C 't, Kozicz Tamas, Nadif Kasri Nael
Abstract excerpt
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is often caused by an adenine to guanine variant at m.3243 (m.3243A>G) of the MT-TL1 gene. To understand how this pathogenic variant affects the nervous system, we differentiated human induced pluripotent stem cells (iPSCs) into excitatory neurons with normal (low heteroplasmy) and impaired (high heteroplasmy) mitochondrial...
Topics
Join the communities discussing this publication.