Article
Generation of induced pluripotent stem (iPS) cells derived from a murine model of Pompe disease and differentiation of Pompe-iPS cells into skeletal muscle cells.
Molecular genetics and metabolism - 1 Jan 2011
Kawagoe Shiho, Higuchi Takashi, Meng Xing-Li, Shimada Yohta, Shimizu Hiromi, Hirayama Reimi, Fukuda Takahiro, Chang Hsi, Nakahata Tatsutoshi, Fukada So-ichiro, Ida Hiroyuki, Kobayashi Hiroshi, Ohashi Toya, Eto Yoshikastu
Abstract excerpt
Our study is the first to demonstrate the ability to generate iPS cells from a mouse model of Pompe disease. Initially, mouse tail tip fibroblasts were harvested from male, 8-week-old (GAA) knockout mice, and three reprogramming factors (Oct3/4, Sox2 and Klf4) were transfected into the isolated donor cells using a retroviral vector. These iPS cells also showed decreased levels of GAA enzymatic activity and strong...
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