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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
immunology and microbiology
Induced pluripotent stem cells: A novel frontier in the study of human primary immunodeficiencies
Journal of Allergy and Clinical Immunology, Volume 127, No. 6, Year 2011
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Description
Background: The novel ability to epigenetically reprogram somatic cells into induced pluripotent stem cells (iPSCs) through the exogenous expression of transcription promises to revolutionize the study of human diseases. Objective: Here we report on the generation of 25 iPSC lines from 6 patients with various forms of primary immunodeficiencies (PIDs) affecting adaptive immunity, innate immunity, or both. Methods: Patients' dermal fibroblasts were reprogrammed by expression of 4 transcription factors, octamer-binding transcription factor 4 (OCT4), sex determining region Y-box 2 (SOX2), Krueppel-like factor 4 (KLF4), and cellular myelomonocytosis proto-oncogene (cMYC), by using a single excisable polycistronic lentiviral vector. Results: iPSCs derived from patients with PIDs show a stemness profile that is comparable with that observed in human embryonic stem cells. After in vitro differentiation into embryoid bodies, pluripotency of the patient-derived iPSC lines was demonstrated by expression of genes characteristic of each of the 3 embryonic layers. We have confirmed the patient-specific origin of the iPSC lines and ascertained maintenance of karyotypic integrity. Conclusion: By providing a limitless source of diseased stem cells that can be differentiated into various cell types in vitro, the repository of iPSC lines from patients with PIDs represents a unique resource to investigate the pathophysiology of hematopoietic and extrahematopoietic manifestations of these diseases and might assist in the development of novel therapeutic approaches based on gene correction. © 2010 American Academy of Allergy, Asthma & Immunology.
Authors & Co-Authors
Pessach, Itai M.
Unknown Affiliation
Ordovás-Montañés, José M.
Unknown Affiliation
Zhang, Shenying
Unknown Affiliation
Casanova, Jean Laurent
Unknown Affiliation
Giliani, S.
Unknown Affiliation
Gennery, Andrew R.
Unknown Affiliation
Al-Herz, Waleed
Unknown Affiliation
Manos, Philip D.
Unknown Affiliation
Schlaeger, Thorsten M.
Unknown Affiliation
Park, In Hyun
Unknown Affiliation
Rucci, Francesca
Unknown Affiliation
Agarwal, Suneet
Unknown Affiliation
Mostoslavsky, Gustavo
Unknown Affiliation
Daley, George Q.
Unknown Affiliation
Notarangelo, Luigi Daniele
Unknown Affiliation
Statistics
Citations: 44
Authors: 15
Affiliations: 12
Identifiers
Doi:
10.1016/j.jaci.2010.11.008
ISSN:
00916749
e-ISSN:
10976825
Research Areas
Cancer
Genetics And Genomics
Health System And Policy