Felsburg Peter J, Hartnett Brian J, Gouthro Terry A, Henthorn Paula S
Department of Clinical Studies, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.
Immunol Res. 2003;27(2-3):235-46. doi: 10.1385/IR:27:2-3:235.
Our laboratory has identified an X-linked severe combined immunodeficiency (XSCID) in dogs that is the result of mutations in the common gamma chain (gammac) subunit of the interleukin-2 (IL-2), IL-4, IL-7, IL-9, IL-15, and IL-21 receptors. Canine XSCID, unlike genetically engineered gammac-deficient mice, has a clinical and immunologic phenotype virtually identical to human XSCID, suggesting species-specific differences exist in the role of the gammac and its associated cytokines in mice in comparison to their role in humans and dogs. This review compares and contrasts thymopoiesis and postnatal T cell development in gammac-deficient (XSCID) dogs raised in a conventional environment, with gammac-deficient dogs raised in a gnotobiotic environment. Therapy to accelerate T cell regeneration following hematopoietic stem cell transplantation or gene therapy is also discussed.
我们实验室在犬类中鉴定出一种X连锁严重联合免疫缺陷(XSCID),它是白细胞介素-2(IL-2)、IL-4、IL-7、IL-9、IL-15和IL-21受体的共同γ链(γc)亚基发生突变的结果。与基因工程γc缺陷小鼠不同,犬类XSCID具有与人类XSCID几乎相同的临床和免疫表型,这表明与在人类和犬类中的作用相比,γc及其相关细胞因子在小鼠中的作用存在物种特异性差异。本综述比较并对比了在常规环境中饲养的γc缺陷(XSCID)犬与在无菌环境中饲养的γc缺陷犬的胸腺生成和出生后T细胞发育。还讨论了造血干细胞移植或基因治疗后加速T细胞再生的治疗方法。