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. 1975 Oct;16(10):796–799. doi: 10.1136/gut.16.10.796

The ultrastructure of hepatocytes in alpha-1-antitrypsin deficiency with the genotype Pi--.

G Feldmann, J P Martin, R Sesboue, C Ropartz, R Perelman, M Nathanson, P Seringe, J P Benhamou
PMCID: PMC1413096  PMID: 1081966

Abstract

The ultrastructural appearance of the endoplasmic reticulum of the hepatocytes was found to be normal in a 5-year-old girl with alpha-1-antitrypsin deficiency with the genotype Pi--. The liver ultrastructure of this variant is therefore different from that of alpha-1-antitrypsin deficiency with the genotype PiZZ in which aggregates of an abnormal, unsecreted alpha-1-antitrypsin accumulate in the endoplasmic reticulum of the hepatocytes. The normal appearance of the endoplasmic reticulum in alpha-1-antitrypsin deficiency with the genotype Pi-- is compatible with the hypothesis, in this variant, synthesis of alpha-1-antitrypsin is completely, or nearly completely, absent; an alternative hypothesis would be that an abnormal alpha-1-antitrypsin is produced by the liver and secreted into the plasma, but disappears rapidly from the plasma.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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