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   induced pluripotent stem cell meets severe combined immunodeficiency  
   
نویسنده kouchaki reza ,abd-nikfarjam bahareh ,maali amirhosein ,abroun saeid ,foroughi farshad ,ghaffari sasan ,azad mehdi
منبع cell journal (yakhteh) - 2020 - دوره : 22 - شماره : Suppl 1 - صفحه:1 -10
چکیده    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.
کلیدواژه hematopoietic stem cell transplantation ,induced pluripotent stem cell ,primary immunodeficiency ,severe combined immunodeficiency
آدرس qazvin university of medical sciences, .faculty of allied medicine, iran, qazvin university of medical sciences, school of medicine, department of immunology, iran, pasteur institute of iran, .student research committee, iran, tarbiat modares university, faculty of medical sciences, department of hematology and blood banking, iran, qazvin university of medical sciences, school of medicine, department of immunology, iran, tehran university of medical sciences, school of allied medicine, hematology department, iran, qazvin university of medical sciences, faculty of allied medicine, iran
 
     
   
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