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Cell Journal، جلد ۲۲، شماره supplement ۱، صفحات ۱-۱۰
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عنوان انگلیسی Induced Pluripotent Stem Cell Meets Severe Combined Immunodeficiency
چکیده انگلیسی مقاله Severe combined immunodeficiency (SCID) is classified as a primary immunodeficiency, which is characterized by impaired T-lymphocytes differentiation. IL2RG, IL7Ralpha, JAK3, ADA, RAG1/RAG2, and DCLE1C (Artemis) are the most defective genes in SCID. The most recent SCID therapies are based on gene therapy (GT) of hematopoietic stem cells (HSC), which are faced with many challenges. The new studies in the field of stem cells have made great progress in overcoming the challenges ahead. In 2006, Yamanaka et al. achieved "reprogramming" technology by introducing four transcription factors known as Yamanaka factors, which generate induced pluripotent stem cells (iPSC) from somatic cells. It is possible to apply iPSC-derived HSC for transplantation in patients with abnormality or loss of function in specific cells or damaged tissue, such as T-cells and NK-cells in the context of SCID. The iPSC-based HSC transplantation in SCID and other hereditary disorders needs gene correction before transplantation. Furthermore, iPSC technology has been introduced as a promising tool in cellular-molecular disease modeling and drug discovery. In this article, we review iPSC-based GT and modeling for SCID disease and novel approaches of iPSC application in SCID.
کلیدواژه‌های انگلیسی مقاله Hematopoietic Stem Cell Transplantation, Induced Pluripotent Stem Cell, Primary Immunodeficiency, Severe Combined Immunodeficiency
نویسندگان مقاله Reza Kouchaki |
Faculty of Allied Medicine, Qazvin University of Medical Sciences, Qazvin, Iran

Bahareh Abd-Nikfarjam |
Department of Immunology, School of Medicine, Qazvin University of Medical Sciences, Qazvin, Iran

Amirhosein Maali |
Student Research Committee, Pasteur Institute of Iran, Tehran, Iran

Saeid Abroun |
Department of Hematology and Blood Banking, Faculty of Medical Sciences, Tarbiat Modares University, Tehran, Iran

Farshad Foroughi |
Department of Immunology, School of Medicine, Qazvin University of Medical Sciences, Qazvin, Iran

Sasan Ghaffari |
Hematology Department, School of Allied Medicine, Tehran University of Medical Sciences, Tehran, Iran

Mehdi Azad |
Faculty of Allied Medicine, Qazvin University of Medical Sciences, Qazvin, Iran

نشانی اینترنتی https://www.celljournal.org/article_250932_906fc32b99a8882201c58b7743262b36.pdf
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