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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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
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spelling doaj-850d5fb426d240efafe9fab322ac92082020-11-24T20:56:58ZengElsevierStem Cell Research1873-50611876-77532017-04-0120C545710.1016/j.scr.2017.02.006Generation of an induced pluripotent stem cell line that mimics the disease phenotypes from a patient with Fanconi anemia by conditional complementationSumitha Prameela Bharathan0Krittika Nandy1Dhavapriya Palani2Nancy Beryl Janet A3Kasthuri Natarajan4Biju George5Alok Srivastava6Shaji Ramachandran Velayudhan7Haematology Department, Christian Medical College, Vellore, Tamil Nadu, IndiaCentre for Stem Cell Research, Christian Medical College, Vellore, Tamil Nadu, IndiaCentre for Stem Cell Research, Christian Medical College, Vellore, Tamil Nadu, IndiaHaematology Department, Christian Medical College, Vellore, Tamil Nadu, IndiaCentre for Stem Cell Research, Christian Medical College, Vellore, Tamil Nadu, IndiaHaematology Department, Christian Medical College, Vellore, Tamil Nadu, IndiaHaematology Department, Christian Medical College, Vellore, Tamil Nadu, IndiaHaematology Department, Christian Medical College, Vellore, Tamil Nadu, IndiaGeneration 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.http://www.sciencedirect.com/science/article/pii/S187350611730020X
collection DOAJ
language English
format Article
sources DOAJ
author Sumitha Prameela Bharathan
Krittika Nandy
Dhavapriya Palani
Nancy Beryl Janet A
Kasthuri Natarajan
Biju George
Alok Srivastava
Shaji Ramachandran Velayudhan
spellingShingle Sumitha Prameela Bharathan
Krittika Nandy
Dhavapriya Palani
Nancy Beryl Janet A
Kasthuri Natarajan
Biju George
Alok Srivastava
Shaji Ramachandran Velayudhan
Generation of an induced pluripotent stem cell line that mimics the disease phenotypes from a patient with Fanconi anemia by conditional complementation
Stem Cell Research
author_facet Sumitha Prameela Bharathan
Krittika Nandy
Dhavapriya Palani
Nancy Beryl Janet A
Kasthuri Natarajan
Biju George
Alok Srivastava
Shaji Ramachandran Velayudhan
author_sort Sumitha Prameela Bharathan
title Generation of an induced pluripotent stem cell line that mimics the disease phenotypes from a patient with Fanconi anemia by conditional complementation
title_short Generation of an induced pluripotent stem cell line that mimics the disease phenotypes from a patient with Fanconi anemia by conditional complementation
title_full Generation of an induced pluripotent stem cell line that mimics the disease phenotypes from a patient with Fanconi anemia by conditional complementation
title_fullStr Generation of an induced pluripotent stem cell line that mimics the disease phenotypes from a patient with Fanconi anemia by conditional complementation
title_full_unstemmed Generation of an induced pluripotent stem cell line that mimics the disease phenotypes from a patient with Fanconi anemia by conditional complementation
title_sort generation of an induced pluripotent stem cell line that mimics the disease phenotypes from a patient with fanconi anemia by conditional complementation
publisher Elsevier
series Stem Cell Research
issn 1873-5061
1876-7753
publishDate 2017-04-01
description 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.
url http://www.sciencedirect.com/science/article/pii/S187350611730020X
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