| Literature DB >> 22704516 |
Shunichi Suzuki1, Masaki Iwamoto2, Yoriko Saito3, Daiichiro Fuchimoto1, Shoichiro Sembon1, Misae Suzuki1, Satoshi Mikawa4, Michiko Hashimoto2, Yuki Aoki3, Yuho Najima3, Shinsuke Takagi3, Nahoko Suzuki3, Emi Suzuki5, Masanori Kubo6, Jun Mimuro7, Yuji Kashiwakura7, Seiji Madoiwa7, Yoichi Sakata7, Anthony C F Perry8, Fumihiko Ishikawa9, Akira Onishi10.
Abstract
A porcine model of severe combined immunodeficiency (SCID) promises to facilitate human cancer studies, the humanization of tissue for xenotransplantation, and the evaluation of stem cells for clinical therapy, but SCID pigs have not been described. We report here the generation and preliminary evaluation of a porcine SCID model. Fibroblasts containing a targeted disruption of the X-linked interleukin-2 receptor gamma chain gene, Il2rg, were used as donors to generate cloned pigs by serial nuclear transfer. Germline transmission of the Il2rg deletion produced healthy Il2rg(+/-) females, while Il2rg(-/Y) males were athymic and exhibited markedly impaired immunoglobulin and T and NK cell production, robustly recapitulating human SCID. Following allogeneic bone marrow transplantation, donor cells stably integrated in Il2rg(-/Y) heterozygotes and reconstituted the Il2rg(-/Y) lymphoid lineage. The SCID pigs described here represent a step toward the comprehensive evaluation of preclinical cellular regenerative strategies.Entities:
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Year: 2012 PMID： 22704516 DOI： 10.1016/j.stem.2012.04.021
Source DB: PubMed Journal: Cell Stem Cell ISSN： 1875-9777 Impact factor: 24.633