Title |
Induced pluripotent stem cells from patients with human fibrodysplasia ossificans progressiva show increased mineralization and cartilage formation
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Published in |
Orphanet Journal of Rare Diseases, December 2013
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DOI | 10.1186/1750-1172-8-190 |
Pubmed ID | |
Authors |
Yoshihisa Matsumoto, Yohei Hayashi, Christopher R Schlieve, Makoto Ikeya, Hannah Kim, Trieu D Nguyen, Salma Sami, Shiro Baba, Emilie Barruet, Akira Nasu, Isao Asaka, Takanobu Otsuka, Shinya Yamanaka, Bruce R Conklin, Junya Toguchida, Edward C Hsiao |
Abstract |
Abnormal activation of endochondral bone formation in soft tissues causes significant medical diseases associated with disability and pain. Hyperactive mutations in the bone morphogenetic protein (BMP) type 1 receptor ACVR1 lead to fibrodysplasia ossificans progressiva (FOP), a rare genetic disorder characterized by progressive ossification in soft tissues. However, the specific cellular mechanisms are unclear. In addition, the difficulty obtaining tissue samples from FOP patients and the limitations in mouse models of FOP hamper our ability to dissect the pathogenesis of FOP. |
X Demographics
Geographical breakdown
Country | Count | As % |
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United Kingdom | 2 | 17% |
Japan | 1 | 8% |
United States | 1 | 8% |
Unknown | 8 | 67% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 10 | 83% |
Scientists | 2 | 17% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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United Kingdom | 1 | <1% |
Sweden | 1 | <1% |
Unknown | 107 | 98% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Ph. D. Student | 23 | 21% |
Researcher | 22 | 20% |
Student > Bachelor | 19 | 17% |
Student > Master | 9 | 8% |
Student > Doctoral Student | 8 | 7% |
Other | 17 | 16% |
Unknown | 11 | 10% |
Readers by discipline | Count | As % |
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Agricultural and Biological Sciences | 33 | 30% |
Medicine and Dentistry | 22 | 20% |
Biochemistry, Genetics and Molecular Biology | 19 | 17% |
Engineering | 4 | 4% |
Nursing and Health Professions | 3 | 3% |
Other | 10 | 9% |
Unknown | 18 | 17% |