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Induction of Pluripotent Stem Cells from a Manifesting Carrier of Duchenne Muscular Dystrophy and Characterization of Their X-Inactivation Status
Joint Authors
Miyagoe-Suzuki, Yuko
Nishiyama, Takashi
Nakamura, Miho
Narita, Asako
Takemura, Fusako
Masuda, Satoru
Minami, Narihiro
Murayama, Kumiko
Komaki, Hirofumi
Goto, Yu-ichi
Takeda, Shin'ichi
Source
Issue
Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-9, 9 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2017-04-12
Country of Publication
Egypt
No. of Pages
9
Abstract EN
Three to eight percent of female carriers of Duchenne muscular dystrophy (DMD) develop dystrophic symptoms ranging from mild muscle weakness to a rapidly progressive DMD-like muscular dystrophy due to skewed inactivation of X chromosomes during early development.
Here, we generated human induced pluripotent stem cells (hiPSCs) from a manifesting female carrier using retroviral or Sendai viral (SeV) vectors and determined their X-inactivation status.
Although manifesting carrier-derived iPS cells showed normal expression of human embryonic stem cell markers and formed well-differentiated teratomas in vivo, many hiPS clones showed bi-allelic expression of the androgen receptor (AR) gene and loss of X-inactivation-specific transcript and trimethyl-histone H3 (Lys27) signals on X chromosomes, suggesting that both X chromosomes of the hiPS cells are in an active state.
Importantly, normal dystrophin was expressed in multinucleated myotubes differentiated from a manifesting carrier of DMD-hiPS cells with XaXa pattern.
AR transcripts were also equally transcribed from both alleles in induced myotubes.
Our results indicated that the inactivated X chromosome in the patient’s fibroblasts was activated during reprogramming, and XCI occurred randomly during differentiation.
American Psychological Association (APA)
Miyagoe-Suzuki, Yuko& Nishiyama, Takashi& Nakamura, Miho& Narita, Asako& Takemura, Fusako& Masuda, Satoru…[et al.]. 2017. Induction of Pluripotent Stem Cells from a Manifesting Carrier of Duchenne Muscular Dystrophy and Characterization of Their X-Inactivation Status. Stem Cells International،Vol. 2017, no. 2017, pp.1-9.
https://search.emarefa.net/detail/BIM-1202193
Modern Language Association (MLA)
Miyagoe-Suzuki, Yuko…[et al.]. Induction of Pluripotent Stem Cells from a Manifesting Carrier of Duchenne Muscular Dystrophy and Characterization of Their X-Inactivation Status. Stem Cells International No. 2017 (2017), pp.1-9.
https://search.emarefa.net/detail/BIM-1202193
American Medical Association (AMA)
Miyagoe-Suzuki, Yuko& Nishiyama, Takashi& Nakamura, Miho& Narita, Asako& Takemura, Fusako& Masuda, Satoru…[et al.]. Induction of Pluripotent Stem Cells from a Manifesting Carrier of Duchenne Muscular Dystrophy and Characterization of Their X-Inactivation Status. Stem Cells International. 2017. Vol. 2017, no. 2017, pp.1-9.
https://search.emarefa.net/detail/BIM-1202193
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1202193