Article
Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.
The Journal of biological chemistry - 25 Nov 2005
Hidvegi Tunda, Schmidt Bela Z, Hale Pamela, Perlmutter David H
Abstract excerpt
In alpha(1)-antitrypsin (alpha1AT) deficiency, a polymerogenic mutant form of the secretory glycoprotein alpha1AT, alpha1ATZ, is retained in the endoplasmic reticulum (ER) of liver cells. It is not yet known how this results in liver injury in a subgroup of deficient individuals and how the remainder of deficient individuals escapes liver disease. One possible explanation is that the "susceptible" subgroup is...
Topics
- Animals
- Caspase 12
- Caspases
- Caspases, Initiator
- Cell Line
- Endoplasmic Reticulum
- Enzyme Activation
- HeLa Cells
- Humans
- Liver
- Membrane Proteins
- Mice
- Mice, Transgenic
- Mutation