Article
Mitochondrial dysfunction and increased reactive oxygen species production in MECP2 mutant astrocytes and their impact on neurons.
Scientific reports - 4 Sept 2024
Tomasello Danielle L, Barrasa M Inmaculada, Mankus David, Alarcon Katia I, Lytton-Jean Abigail K R, Liu X Shawn, Jaenisch Rudolf
Abstract excerpt
Studies on MECP2 function and its implications in Rett Syndrome (RTT) have traditionally centered on neurons. Here, using human embryonic stem cell (hESC) lines, we modeled MECP2 loss-of-function to explore its effects on astrocyte (AST) development and dysfunction in the brain. Ultrastructural analysis of RTT hESC-derived cerebral organoids revealed significantly smaller mitochondria compared to controls (CTRs),...
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