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

Muhammad Arsalan Iqbal, Kwonho Hong, Jin Hoi Kim, Youngsok Choi
2019 BMB Reports  
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
more » ... ll−/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. [BMB Reports 2019; 52(11): 625-634] X-LINKED-SCID BY IL2RG MUTATIONS: T−B+NK− This type of SCID is generally caused by gene mutation that affects the integrity between IL2RG and JAK3 signaling pathway. The most common form of SCID in humans is the X-linked recessive SCID (T−B+NK−). The gamma chain is a BMB Rep. 2019; 52(11): 625-634 Invited Mini Review Role of SCID pig model in biomedical research Muhammad Arsalan Iqbal, et al.
doi:10.5483/bmbrep.2019.52.11.267 fatcat:fjbeedfd3vemra6fn52y6tmkbe