Stem cell modeling of mitochondrial parkinsonism reveals key functions of OPA1.

Mindaugas JonikasMartin MadillAlexandre MathyTheresa ZekollChristos E ZoisSimon WigfieldMarzena Kurzawa-AkanbiCathy BrowneDavid SimsPatrick F ChinnerySally A CowleyGeorge K Tofaris

Published in: Annals of neurology (2018)

OPA1 levels maintain oxidative phosphorylation in iPSC-derived neurons, at least in part, by regulating the stability of complex I. Severity of OPA1 disease associates primarily with the extent of OPA1-mediated fusion, suggesting that activation of this mechanism or identification of its genetic modifiers may have therapeutic or prognostic value. Ann Neurol 2018;83:915-925.

Keyphrases

stem cells
oxidative stress
spinal cord
genome wide
dna methylation
copy number
mesenchymal stem cells
drug induced
bone marrow