Accumulation of Mutant α1-Antitrypsin Z in the Endoplasmic Reticulum Activates Caspases-4 and -12, NFκB, and BAP31 but Not the Unfolded Protein Response

@inproceedings{Hidvegi2005AccumulationOM,
  title={Accumulation of Mutant α1-Antitrypsin Z in the Endoplasmic Reticulum Activates Caspases-4 and -12, NFκB, and BAP31 but Not the Unfolded Protein Response},
  author={Tunda Hidvegi and Bela Z. Schmidt and Pamela J. Hale and David H. Perlmutter},
  year={2005}
}
Abstract In α1-antitrypsin (α1AT) deficiency, a polymerogenic mutant form of the secretory glycoprotein α1AT, α1ATZ, 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 unable to mount the appropriate protective cellular responses. Here we examined the effect of… CONTINUE READING

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