Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z alpha 1-antitrypsin deficiency.

Abstract

Conformational diseases are a class of disorders associated with aberrant protein accumulation in tissues and cellular compartments. Z alpha1-antitrypsin (A1AT) deficiency is a genetic disease associated with accumulation of misfolded A1AT in the endoplasmic reticulum (ER) of hepatocytes. We sought to identify intracellular events involved in the molecular… (More)

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@article{Lawless2004ActivationOE, title={Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z alpha 1-antitrypsin deficiency.}, author={Matthew W. Lawless and Catherine M. Greene and Alan T. Mulgrew and Clifford C. Taggart and Shane O'Neill and Noel Gerard McElvaney}, journal={Journal of immunology}, year={2004}, volume={172 9}, pages={5722-6} }