ISSN:
1365-2559
Source:
Blackwell Publishing Journal Backfiles 1879-2005
Topics:
Medicine
Notes:
We present a case of chronic liver disease with selective and exclusive hepatocyte endoplasmic reticulum storage of δ1-antichymotrypsin in the form of granules, detected by specific immunohistochemistry at the light microscopy level and corresponding to material found in dilated endoplasmic reticulum of hepatocytes by electron microscopy. The patient had intermediate deficiency of δ1-antichymotrypsin. Thus, the hepatocyte accumulation of δ1-antichymotrypsin may indicate the presence of an export block resembling that of a closely-related protein, namely PiZ δ1-antitrypsin. It is proposed that hepatocyte storage of δ1-antichymotrypsin may be an expression of an inborn error of metabolism bearing the characteristics of endoplasmic reticulum storage diseases such as PiZ δ1-antitrypsin deficiency and hereditary hypofibrinogenaemia.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1111/j.1365-2559.1990.tb01107.x
