PubMed Health⌕ Search

Biomedical subjects

P Seringe

Publications and source records attributed to P Seringe.

At least 19 recordsLinked to original sources

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

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.

Carbohydrate Metabolism, Inborn Errors↗

[Drepanocytosis].

Explore the source record for details and available documents.

Abdomen, Acute↗