Fudenberg H H, Goust J M, Vesole D H, Salinas C F
Gerontology. 1979;25(4):231-7. doi: 10.1159/000212345.
Active, total and nonspecific suppressor T cells were studied in a 15-year-old black male with dyskeratosis congenita syndrome, a precancerous mucosal disease, and in 7 siblings and several other relatives in three generations. The propositus and 1 elder sister, products of a second-cousin marriage, died with dyskeratosis congenita. The mother had dermatomyositis, and the maternal grandmother and her sister reportedly had rheumatoid arthritis. Studies of available siblings, father, and grandparents revealed a high incidence of deficiency in number of active and/or suppressor T cells, sometimes severe enough to result in a decrease in total T cells. The patient had many stigmata of precocious aging, as did the sibling who died with the same syndrome. The laboratory data suggest that a defect in cell-mediated immunity, involving mainly or exclusively suppressor T cells, is associated with, and is presumably the cause of, precocious aging; perhaps an abiotrophy in this cell subpopulation results in physiologic aging.
对一名患有先天性角化不良综合征(一种癌前黏膜疾病)的15岁黑人男性以及其三代内的7名兄弟姐妹和其他几名亲属进行了活性、总抑制性和非特异性抑制性T细胞研究。先证者和一名表姐婚姻所生的姐姐死于先天性角化不良。母亲患有皮肌炎,据报道外祖母及其妹妹患有类风湿性关节炎。对在世的兄弟姐妹、父亲和祖父母的研究显示,活性和/或抑制性T细胞数量不足的发生率很高,有时严重到足以导致总T细胞数量减少。该患者有许多早衰的体征,死于相同综合征的兄弟姐妹也是如此。实验室数据表明,主要或仅涉及抑制性T细胞的细胞介导免疫缺陷与早衰相关,并且可能是早衰的原因;也许这个细胞亚群的营养缺陷导致了生理衰老。