Mitochondrial Disease: mtDNA and Protein Segregation Mysteries in iPSCs

摘要

Mitochondrial diseases cause a range of clinical manifestations even in patients carrying the same mtDNA mutations. New work reveals that a common disease-associated mtDNA mutation is selectively segregated from wild-type mtDNA during the reprogramming of induced pluripotent stem cells and that high levels of this mutation in differentiated neurons upregulate Parkin-mediated mitophagy.