Identification of PiZ gene products in liver tissue by a monoclonal antibody specific for the Z mutant of α1-antitrypsin

Francesco Callea, Massimo Brisigotti, Gavino Faa, Laura Lucini, Sten Eriksson

Research output: Contribution to journalArticle

Abstract

Globular inclusions of abnormal α1-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 hepatocytotes, 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.

Original languageEnglish
Pages (from-to)372-376
Number of pages5
JournalJournal of Hepatology
Volume12
Issue number3
DOIs
Publication statusPublished - 1991

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ASJC Scopus subject areas

  • Gastroenterology

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