Callea F, Brisigotti M, Faa G, Lucini L, Eriksson S
Department of Pathology, Istituto G. Gaslini, Genova, Italy.
J Hepatol. 1991 May;12(3):372-6. doi: 10.1016/0168-8278(91)90842-y.
Globular inclusions of abnormal alpha 1-antitrypsin (AAT) in the rough endoplasmic reticulum of hepatocytes is a characteristic feature of AAT deficiency of the PiZ phenotype. It is also seen in some rare M-like Pi types (including M-Cagliari) having low plasma AAT levels and M-like mobility on isoelectric focusing. In this report the ability of a monoclonal antibody (ATZ 11) raised against PiZ hepatocytic AAT to identify AAT inclusions by immunohistochemical techniques is evaluated. The antibody was found to specifically and selectively identify the PiZ gene products in hepatocytes, but not M-Cagliari AAT. Application of the method thus allows distinction of PiZ gene carriers from PiM-like subjects in the absence of serum protein analysis.
肝细胞粗面内质网中异常α1-抗胰蛋白酶（AAT）的球状包涵体是PiZ表型AAT缺乏症的特征性表现。在一些血浆AAT水平低且在等电聚焦时有M样迁移率的罕见M样Pi类型（包括M-卡利亚里型）中也可见到。在本报告中，评估了一种针对PiZ肝细胞AAT产生的单克隆抗体（ATZ 11）通过免疫组织化学技术识别AAT包涵体的能力。发现该抗体能特异性且选择性地识别肝细胞中的PiZ基因产物，但不能识别M-卡利亚里型AAT。因此，在不进行血清蛋白分析的情况下，应用该方法可区分PiZ基因携带者和PiM样个体。
