Premium
Storage of alpha‐1‐antitrypsin in intrahepatic bile duct cells in alpha‐1‐antitrypsin deficiency (Pi Z phenotype)
Author(s) -
CALLEA F.,
FEVERY J.,
MASSI G.,
DE GROOTE J.,
DESMET V. J.
Publication year - 1985
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/j.1365-2559.1985.tb02973.x
Subject(s) - alpha 1 antitrypsin deficiency , intrahepatic bile ducts , bile duct , pathology , phenotype , cirrhosis , alpha (finance) , hyperplasia , biology , intracellular , medicine , chemistry , gene , biochemistry , nursing , patient satisfaction , construct validity
Storage of alpha‐1‐antitrypsin (AAT) has been found in a small number of bile duct cells in liver tissue specimens from patients with Pi MZ, Pi SZ and Pi ZZ phenotypes. The storage appeared in the form of intracellular AAT immunoreactive inclusions. On EM investigation, AAT‐like material was detected within cisternae of the RER and SER. Such AAT inclusions were found in proliferating bile ductules in conditions such as cirrhosis, focal nodular hyperplasia and extrahepatic obstruction. They were also observed in normal biliary structures at the level of the canals of Hering, bile ductules and interlobular ducts in 13 out of 47 cases. These findings are interpreted as indicating that the intrahepatic bile duct cells are a further source of AAT, and that in case of defective export of AAT from the cell, as is the case for the Z protein, the protein accumulates not only in hepatocytes but in biliary cells as well.