Godfrey V L, Wilkinson J E, Rinchik E M, Russell L B
Biology Division, Oak Ridge National Laboratory, TN 37831-8077.
Proc Natl Acad Sci U S A. 1991 Jul 1;88(13):5528-32. doi: 10.1073/pnas.88.13.5528.
Characteristic lesions in mice hemi- or homozygous for the X-linked mutation scurfy (sf) include lymphohistiocytic proliferation in the skin and lymphoid organs, Coombs' test-positive anemia, hypergammaglobulinemia, and death by 24 days of age. The role of the thymus in the development of fatal lymphoreticular disease in the scurfy mouse was investigated. Neonatal thymectomy doubles the life span of scurfy mice, moderates the histologic lesions, and prevents anemia, despite the continued presence of high levels of serum IgG. Animals bred to be nude and scurfy (nu/nu; sf/Y) are viable, fertile, and free of scurfy lesions. Bone marrow from scurfy mice can reconstitute lethally irradiated, H-2-compatible animals but does not transmit scurfy disease. We conclude, from these data, that scurfy lesions are mediated by T lymphocytes that mature in an abnormal (sf) thymic environment.
X连锁突变“鳞屑”（sf）的半合子或纯合子小鼠的特征性病变包括皮肤和淋巴器官中的淋巴细胞组织细胞增生、库姆斯试验阳性贫血、高球蛋白血症，以及在24日龄前死亡。研究了胸腺在鳞屑小鼠致命性淋巴网状疾病发展中的作用。新生小鼠胸腺切除使鳞屑小鼠的寿命延长一倍，减轻了组织学病变，并预防了贫血，尽管血清IgG水平持续升高。培育出的裸鼠和鳞屑鼠（nu/nu；sf/Y）是有活力的、可育的，且没有鳞屑病变。来自鳞屑小鼠的骨髓可以重建经致死性照射且H-2相容的动物，但不会传播鳞屑病。根据这些数据，我们得出结论，鳞屑病变是由在异常（sf）胸腺环境中成熟的T淋巴细胞介导的。
