ASSESSMENT OF PERIPHERAL BLOOD MONOCYTES IN PATIENTS WITH RECURRENT MYCOBACTERIAL

单核细胞的特征是CD40、CD80、CD86、CD120b和γ干扰素受体α链的表达水平。这些研究已经确定了两例γ干扰素受体α链缺乏的患者，并且正在积极检查这种缺陷的分子水平。此外，1例干扰素γ受体β链缺乏症患者也在分子水平上进行了表征。作为这些研究的结果，一种用于评估STAT蛋白磷酸化的细胞内流式细胞术方法已经被开发和验证。这种方法正在研究各种各样的激活相关蛋白改变，似乎可用于筛查由共同γ链和JAK3缺陷引起的严重联合免疫缺陷患者。

Monocytes are being characterized for the expression levels of CD40, CD80, CD86, CD120b, and the gamma interferon receptor alpha chain. These studies have identified two patients with gamma interferon receptor alpha chain deficiency, and the molecu-lar level of this defect is being actively examined. In addition, one patient with interferon gamma receptor beta chain deficiency also has been characterized at the molecular level. As a consequence of these studies, an intracellular flow cytometric method for evaluating STAT protein phosphorylation has been developed and validated. This approach is being investigated for a wide variety of activation associated protein alterations and appears to be useful in screening for patients with severe combined immunodeficiency due to defects in the common gamma chain and JAK3.