title: Reprogramming efficiency and quality of induced Pluripotent Stem Cells (iPSCs) generated from muscle-derived fibroblasts of mdx mice at different ages.
abstract: Induced pluripotent stem cells (iPSCs) hold promise as a potential treatment for Duchenne muscular dystrophy (DMD). To determine the impact of the donor's age on reprogramming, we generated iPSCs from muscle-derived fibroblasts (MuFs) of mdx mice aged 6 weeks, 6 months, and 14 months. MuFs from 14-month-old mdx mice showed lower proliferative activity and lower reprogramming efficiency, compared with those from younger mdx mice. Furthermore, iPSCs derived from 14-month-old mdx mice (14m-MuF-iPSCs) gradually lost Nanog expression, and regressed in conventional ES medium during passages. Interestingly, inhibition of TGF-β signaling and BMP signaling stabilized Nanog expression and promoted self-renewal of 14m-MuF-iPSCs. Finally, rescued mdx-derived iPSCs efficiently differentiated into the skeletal muscle lineage.
authors.affiliation: Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-higashi, Kodaira, Tokyo 187-8502, Japan;  1.Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira 187-8502, Japan 2.Department of Biological Information, Tokyo Institute of Technology, Yokohama 226-8501, Japan and  1Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-higashi, Kodaira, Tokyo 187-8502, Japan 2Department of Life Sciences, The University of Tokyo, 3-8-1 Komaba, Tokyo 153-890, Japan.
authors.forename: Bo
authors.initials: B
authors.lastname: Wang
authors.forename: Yuko
authors.initials: Y
authors.lastname: Miyagoe-Suzuki
authors.forename: Erica
authors.initials: E
authors.lastname: Yada
authors.forename: Naoki
authors.initials: N
authors.lastname: Ito
authors.forename: Takashi
authors.initials: T
authors.lastname: Nishiyama
authors.forename: Miho
authors.initials: M
authors.lastname: Nakamura
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Ono
authors.forename: Norio
authors.initials: N
authors.lastname: Motohashi
authors.forename: Makoto
authors.initials: M
authors.lastname: Segawa
authors.forename: Satoru
authors.initials: S
authors.lastname: Masuda
authors.forename: Shin'ichi
authors.initials: S
authors.lastname: Takeda
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