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首页> 外文期刊>Cytometry: The Journal of the Society for Analytical Cytology >Investigation of suspected deficient Fas-mediated apoptosis in a father and son
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Investigation of suspected deficient Fas-mediated apoptosis in a father and son

机译：父子疑似Fas介导的细胞凋亡不足的调查

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Background: A 2-year-old boy presented with symptoms consistent with a diagnosis of autoimmune lymphoproliferative syndrome (ALPS), His father had been splenectomized at age 12 with similar symptoms. ALPS is a rare hereditary syndrome that may result from a functional defect in Fas-mediated apoptosis. Methods: Peripheral blood lymphocytes (PBL) and splenic lymphocytes from the patient and PBL from his father and a normal control were analyzed for surface Fas expression. They were then stimulated with an anti-Fas monoclonal antibody (DX2), Apoptosis was assayed by flow cytometry at 0, 20, 28, and 34 h. Results: There was no significant difference in expression of Fas (CD95) in the PBL of the patient. his father, or the normal control, or the splenic lymphocytes. Compared with the normal control, the PBL of the patient and his father failed to progress to apoptosis. They also contained a markedly elevated proportion of CD3+CD4-CD8-"double-negative" cells. Conclusions: PBL from both the patient and his father expressed CD95, but failed to proceed to apoptosis after stimulation, suggesting a functional defect. These results and the clinical presentation are consistent with published descriptions of ALPS. (C) 2001 Wiley-Liss, Inc. [References: 17]
机译：背景：一个2岁男孩的症状与自身免疫性淋巴组织增生综合征（ALPS）的诊断相符，他的父亲在12岁时被脾切除并出现了类似症状。 ALPS是一种罕见的遗传综合征，可能是由Fas介导的细胞凋亡的功能缺陷引起的。方法：分析患者外周血淋巴细胞（PBL）和脾淋巴细胞以及其父亲和正常对照的外周血淋巴细胞表面Fas表达。然后将它们用抗Fas单克隆抗体（DX2）刺激，在0、20、28和34小时通过流式细胞仪检测细胞凋亡。结果：患者的PBL中Fas（CD95）的表达没有显着差异。他的父亲，或正常对照，或脾淋巴细胞。与正常对照组相比，患者及其父亲的PBL未能进展为细胞凋亡。它们还含有明显升高比例的CD3 + CD4-CD8-“双阴性”细胞。结论：患者和父亲的PBL均表达CD95，但在刺激后未能继续凋亡，提示功能缺陷。这些结果和临床表现与已发表的ALPS描述一致。 （C）2001 Wiley-Liss，Inc. [参考：17]

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