[HTML][HTML] The proteasome participates in degradation of mutant α1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes
JH Teckman, J Burrows, T Hidvegi, B Schmidt… - Journal of Biological …, 2001 - ASBMB
Because retention of mutant α 1-antitrypsin (α 1-AT) Z in the endoplasmic reticulum (ER) is
associated with liver disease in α 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 involvement of the proteasome in degradation
of α 1-ATZ (Qu, D., Teckman, JH, Omura, S., and Perlmutter, DH (1996) J. Biol. Chem. 271 …
associated with liver disease in α 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 involvement of the proteasome in degradation
of α 1-ATZ (Qu, D., Teckman, JH, Omura, S., and Perlmutter, DH (1996) J. Biol. Chem. 271 …