Impaired respiratory function in MELAS-induced pluripotent stem cells with high heteroplasmy levels

•We modeled the mitochondrial disease MELAS by generating patient-specific iPS cells.•MELAS-iPS cells show a wide variety of heteroplasmy levels.•MELAS-iPS cells with high heteroplasmy levels showed impaired complex I activity.

Bibliographic Details
Main Authors: Kodaira, Masaki, Hatakeyama, Hideyuki, Yuasa, Shinsuke, Seki, Tomohisa, Egashira, Toru, Tohyama, Shugo, Kuroda, Yusuke, Tanaka, Atsushi, Okata, Shinichiro, Hashimoto, Hisayuki, Kusumoto, Dai, Kunitomi, Akira, Takei, Makoto, Kashimura, Shin, Suzuki, Tomoyuki, Yozu, Gakuto, Shimojima, Masaya, Motoda, Chikaaki, Hayashiji, Nozomi, Saito, Yuki, Goto, Yu-ichi, Fukuda, Keiichi
Format: Online
Language:English
Published: Elsevier 2015
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4383791/