Article
The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes.
The Journal of biological chemistry - 30 Nov 2001
Teckman J H, Burrows J, Hidvegi T, Schmidt B, Hale P D, Perlmutter D H
Abstract excerpt
Because retention of mutant alpha(1)-antitrypsin (alpha(1)-AT) Z in the endoplasmic reticulum (ER) is associated with liver disease in alpha(1)-AT-deficient individuals, the mechanism by which this aggregated glycoprotein is degraded has received considerable attention. In previous studies using stable transfected human fibroblast cell lines and a cell-free microsomal translocation system, we found evidence for...
Topics
- Acetylcysteine
- Animals
- Antibiotics, Antineoplastic
- Carcinoma, Hepatocellular
- Cell Line
- Cells, Cultured
- Cysteine Endopeptidases
- Electrophoresis, Polyacrylamide Gel
- Endoplasmic Reticulum
- Fibroblasts