NDLI: Selenoprotein S/SEPS1 Modifies Endoplasmic Reticulum Stress in Z Variant α1-Antitrypsin Deficiency*

Content Provider	PubMed Central
Author	Kelly, Emer Greene, Catherine M. Carroll, Tomás P. Mcelvaney, Noel G. O'neill, Shane J.
Abstract	Z α1-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 ZAAT transgene. We evaluated levels of NFκB activity as a marker of the ER overload response. To determine the effect of selenium supplementation on the function of SEPS1, we investigated glutathione peroxidase activity, grp78 promoter activity, and NFκB activity in the presence or absence of selenium. SEPS1 reduced levels of active ATF6. Overexpression of SEPS1 also inhibited grp78 promoter and NFκB activity, and this effect was enhanced in the presence of selenium supplementation. This finding demonstrates a role for SEPS1 in ZAAT deficiency and suggests a possible therapeutic potential for selenium supplementation.
Related Links	http://dx.doi.org/10.1074/jbc.m109.006288
Ending Page	16897
Page Count	7
Starting Page	16891
File Format	PDF
ISSN	00219258
e-ISSN	1083351X
Journal	The Journal of Biological Chemistry
Issue Number	25
Volume Number	284
Language	English
Publisher	American Society for Biochemistry and Molecular Biology
Publisher Date	2009-06-19
Access Restriction	Open
Rights Holder	American Society for Biochemistry and Molecular Biology
Subject Keyword	Cell Biology Biochemistry Molecular Biology Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Cell Biology Biochemistry Molecular Biology

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Selenoprotein S/SEPS1 Modifies Endoplasmic Reticulum Stress in Z Variant α1-Antitrypsin Deficiency

Selenoprotein S/SEPS1 modifies endoplasmic reticulum stress in Z variant alpha1-antitrypsin deficiency.

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Endoplasmic reticulum polymers impair luminal protein mobility and sensitise to cellular stress in α1-antitrypsin deficiency

Selenoprotein S/SEPS1 Modifies Endoplasmic Reticulum Stress in Z Variant α1-Antitrypsin Deficiency*

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Selenoprotein S/SEPS1 Modifies Endoplasmic Reticulum Stress in Z Variant α1-Antitrypsin Deficiency

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