Generation of an induced pluripotent stem cell line that mimics the disease phenotypes from a patient with Fanconi anemia by conditional complementation

Generation of an induced pluripotent stem cell line that mimics the disease phenotypes from a patient with Fanconi anemia by conditional complementation

Generation of Fanconi anemia (FA) patient-specific induced pluripotent stem cells (iPSCs) has been reported to be technically challenging due to the defects in the FA-pathway in the patients' somatic cells. By inducible complementation of FA-pathway, we successfully reprogrammed the fibroblasts...

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Bibliographic Details
Main Authors: Sumitha Prameela Bharathan, Krittika Nandy, Dhavapriya Palani, Nancy Beryl Janet A, Kasthuri Natarajan, Biju George, Alok Srivastava, Shaji Ramachandran Velayudhan
Format: Article
Language:English
Published: Elsevier 2017-04-01
Series:Stem Cell Research
Online Access:http://www.sciencedirect.com/science/article/pii/S187350611730020X
Description
Summary:Generation of Fanconi anemia (FA) patient-specific induced pluripotent stem cells (iPSCs) has been reported to be technically challenging due to the defects in the FA-pathway in the patients' somatic cells. By inducible complementation of FA-pathway, we successfully reprogrammed the fibroblasts of an FA patient to iPSCs. CSCR19i-indCFANCA, one of the iPSC lines generated by the inducible complementation of FA-pathway, was extensively characterized for its pluripotency and karyotype. In the absence of doxycycline (DOX) and FANCA expression, this line showed the cellular phenotypes of FA, suggesting it is an excellent tool for FA disease modeling and drug screening.
ISSN:1873-5061
1876-7753

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