Hepatocyte inclusion of α1-antichymotrypsin in a patient with partial deficiency of α1-antichymotrypsin and chronic liver disease

B. Lindmark, H. Millward-Sadler, F. Callea, S. Eriksson

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

We present a case of chronic liver disease with selective and exclusive hepatocyte endoplasmic reticulum storage of α1-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 α1-antichymotrypsin. Thus, the hepatocyte accumulation of α1-antichymotrypsin may indicate the presence of an export block resembling that of a closely-related protein, namely PiZ α1-antitrypsin. It is proposed that hepatocyte storage of α1-antichymotrypsin may be an expression of an inborn error of metabolism bearing the characteristics of endoplasmic reticulum storage diseases such as PiZ α1-antitrypsin deficiency and hereditary hypofibrinogenaemia.

Original languageEnglish
Pages (from-to)221-225
Number of pages5
JournalHistopathology
Volume16
Issue number3
Publication statusPublished - 1990

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Liver Diseases
Hepatocytes
Chronic Disease
Endoplasmic Reticulum
Inborn Errors Metabolism
Microscopy
Electron Microscopy
Immunohistochemistry
Light
Proteins

Keywords

  • α-antichymotrypsin
  • α-antitrypsin
  • Chronic active hepatitis
  • Endoplasmic reticulum
  • Fibrinogen
  • Liver

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Cell Biology

Cite this

Hepatocyte inclusion of α1-antichymotrypsin in a patient with partial deficiency of α1-antichymotrypsin and chronic liver disease. / Lindmark, B.; Millward-Sadler, H.; Callea, F.; Eriksson, S.

In: Histopathology, Vol. 16, No. 3, 1990, p. 221-225.

Research output: Contribution to journalArticle

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