[HTML][HTML] Selenoprotein S/SEPS1 modifies endoplasmic reticulum stress in Z variant α1-antitrypsin deficiency

E Kelly, CM Greene, TP Carroll, NG McElvaney… - Journal of biological …, 2009 - ASBMB
Z α 1-antitrypsin (ZAAT) deficiency is a disease associated with emphysematous lung
disease and also with liver disease. The liver disease of AAT deficiency is associated with
endoplasmic reticulum (ER) stress. SEPS1 is a selenoprotein that, through a chaperone
activity, decreases ER stress. To determine the effect of SEPS1 on ER stress in ZAAT
deficiency, we measured activity of the grp78 promoter and levels of active ATF6 as markers
of the unfolded protein response in HepG2 cells transfected with the mutant form of AAT, a …