Il2rg Gene-Targeted Severe Combined Immunodeficiency Pigs

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• 作者：Shunichi Suzuki¹ ; Masaki Iwamoto³ ; Yoriko Saito⁴ ; Daiichiro Fuchimoto¹ ; Shoichiro Sembon¹ ; Misae Suzuki¹ ; Satoshi Mikawa² ; Michiko Hashimoto³ ; Yuki Aoki⁴ ; Yuho Najima⁴ ; Shinsuke Takagi⁴ ; Nahoko Suzuki⁴ ; Emi Suzuki⁵ ; Masanori Kubo⁶ ; Jun Mimuro⁷ ; Yuji Kashiwakura⁷ ; Seiji Madoiwa⁷ ; Yoichi Sakata⁷ ; Anthony  ; C.F. Perry⁸ ; Fumihiko Ishikawa⁴ ; ^{f_ishika@rcai.riken.jp} ; Akira Onishi¹ ; ^{onishi@affrc.go.jp}
• 刊名：Cell Stem Cell
• 出版年：2012
• 期刊代码：P15_19345909
• 类别：bio
• 出版时间：14 June, 2012
• 卷：10
• 期：6
• 页码：753-758
• 文件大小：2385 K

摘要

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Summary

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^+/鈭?/sup> females, while Il2rg^鈭?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^鈭?Y heterozygotes and reconstituted the Il2rg^鈭?Y lymphoid lineage. The SCID pigs described here represent a step toward the comprehensive evaluation of preclinical cellular regenerative strategies.