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http://dx.doi.org/10.5483/BMBRep.2019.52.11.267

Severe combined immunodeficiency pig as an emerging animal model for human diseases and regenerative medicines  

Iqbal, Muhammad Arsalan (Department of Stem Cell and Regenerative Biotechnology, Humanized Pig Research Center (SRC), Konkuk University)
Hong, Kwonho (Department of Stem Cell and Regenerative Biotechnology, Humanized Pig Research Center (SRC), Konkuk University)
Kim, Jin Hoi (Department of Stem Cell and Regenerative Biotechnology, Humanized Pig Research Center (SRC), Konkuk University)
Choi, Youngsok (Department of Stem Cell and Regenerative Biotechnology, Humanized Pig Research Center (SRC), Konkuk University)
Publication Information
BMB Reports / v.52, no.11, 2019 , pp. 625-634 More about this Journal
Abstract
Severe combined immunodeficiency (SCID) is a group of inherited disorders characterized by compromised T lymphocyte differentiation related to abnormal development of other lymphocytes [i.e., B and/or natural killer (NK) cells], leading to death early in life unless treated immediately with hematopoietic stem cell transplant. Functional NK cells may impact engraftment success of life-saving procedures such as bone marrow transplantation in human SCID patients. Therefore, in animal models, a T cell-/B cell-/NK cell＋ environment provides a valuable tool for understanding the function of the innate immune system and for developing targeted NK therapies against human immune diseases. In this review, we focus on underlying mechanisms of human SCID, recent progress in the development of SCID animal models, and utilization of SCID pig model in biomedical sciences. Numerous physiologies in pig are comparable to those in human such as immune system, X-linked heritability, typical T-B＋NK- cellular phenotype, and anatomy. Due to analogous features of pig to those of human, studies have found that immunodeficient pig is the most appropriate model for human SCID.
Keywords
Genetic mutations; Pig immunodeficient model; Regenerative medicine; SCID; Xenotransplantation;
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