Absence of typical unfolded protein response in primary cultured cystic fibrosis airway epithelial cells

Biochemical and Biophysical Research Communications
Suparna NanuaMarc B Hershenson

Abstract

We examined whether the unfolded protein response is activated in cells expressing incorrectly folded cystic fibrosis transmembrane conductance regulator. Airway epithelial cells from three control and three CF patients homozygous for the deltaF508 mutation were tested. There were no differences in protein expression of the pro-apoptotic factor C/EBP homologous protein (CHOP) or the endoplasmic reticulum (ER) chaperone binding Ig protein. Nor were there differences in phosphorylation of protein kinase R-like ER kinase or eukaryotic initiation factor-2alpha, or the splicing of X-box binding protein (XBP)-1. However, CF cells showed increased mRNA expression of CHOP and XBP-1. A proteasome inhibitor increased CHOP expression in CF cells, suggesting that enhanced proteasome activation is responsible for the observed post-transcriptional regulation. Finally, CF cells were resistant to apoptosis, suggesting that post-transcriptional regulation of CHOP prevents apoptosis. While CHOP and XBP-1 mRNA expression is increased in CF cells, the classic UPR is not present.

References

Apr 1, 1991·American Journal of Respiratory Cell and Molecular Biology·P L ZeitlinW B Guggino
Nov 1, 1989·American Journal of Respiratory Cell and Molecular Biology·R N SifersS L Woo
Sep 1, 1995·The Journal of Clinical Investigation·F DupuitE Puchelle
Oct 6, 1995·Cell·C L WardR R Kopito
Oct 15, 1993·Proceedings of the National Academy of Sciences of the United States of America·Y YangJ M Wilson
Apr 16, 1996·Proceedings of the National Academy of Sciences of the United States of America·R A Gottlieb, A Dosanjh
Mar 17, 1999·The EMBO Journal·G C MeachamD M Cyr
Feb 21, 2002·The Journal of Clinical Investigation·Seiichi OyadomariMasataka Mori
Oct 22, 2002·American Journal of Physiology. Lung Cellular and Molecular Physiology·Jing LiMarc B Hershenson
Aug 6, 2003·Trends in Neurosciences·Mark S FormanJohn Q Trojanowski
Apr 22, 2004·The Journal of Immunology : Official Journal of the American Association of Immunologists·Matthew W LawlessNoel G McElvaney
Mar 5, 2005·The Journal of Biological Chemistry·Carla M Pedrosa RibeiroRichard C Boucher
Jan 15, 2008·Journal of Inherited Metabolic Disease·C M GreeneN G McElvaney

❮ Previous
Next ❯

Citations

Mar 27, 2009·The Journal of Biological Chemistry·Mary E B MartinoCarla M P Ribeiro
Nov 18, 2008·American Journal of Respiratory Cell and Molecular Biology·Luc DannhofferThierry Chinet
May 7, 2008·American Journal of Respiratory Cell and Molecular Biology·Rafal BartoszewskiZsuzsa Bebok
Oct 30, 2010·Proceedings of the American Thoracic Society·Carla M P Ribeiro, Richard C Boucher
Oct 18, 2011·Journal of Inherited Metabolic Disease·Guglielmo R D VillaniPaola Di Natale
May 1, 2007·Biochemical Pharmacology·Catherine VerhaegheVincent Bours
Nov 7, 2015·Molecular and Cellular Pediatrics·Irene K OglesbyCatherine M Greene
Apr 2, 2014·The International Journal of Biochemistry & Cell Biology·Iwona M Pranke, Isabelle Sermet-Gaudelus
Nov 21, 2007·Biochimica Et Biophysica Acta·Mathieu KerbiriouPascal Trouvé
Oct 5, 2016·Cellular and Molecular Life Sciences : CMLS·Carlos M Farinha, Sara Canato
Oct 28, 2019·Journal of Biophotonics·Giuseppe BellisolaGianfelice Cinque
Nov 29, 2012·Indian Journal of Critical Care Medicine : Peer-reviewed, Official Publication of Indian Society of Critical Care Medicine·Umesh GoneppanavarSouvik Chaudhury
Jun 17, 2020·Clinical Science·Brahmchetna BediRuxana T Sadikot
Oct 30, 2012·The Journal of Immunology : Official Journal of the American Association of Immunologists·Christoph J BlohmkeStuart E Turvey

❮ Previous
Next ❯

Related Concepts

Related Feeds

Alzheimer's Disease: Transcription

Impaired transcription is associated with the pathogenesis and progression of conditions such as Alzheimer's disease (AD). Here are the latest discoveries pertaining to transcription and AD.

Alzheimer's Disease: APP

Amyloid precursor protein (APP) proteolysis is critical for the development of Alzheimer's disease, a neurodegenerative disease associated with accumulation of amyloid plaques in the brain. Here is the latest research on APP and Alzheimer's disease.

Alzheimer's Disease: Amyloid Beta

Alzheimer's disease is a neurodegenerative disease associated with the accumulation of amyloid plaques in the brain; these plaques are comprised of amyloid beta deposits. Here is the latest research in this field.

Apoptosis

Apoptosis is a specific process that leads to programmed cell death through the activation of an evolutionary conserved intracellular pathway leading to pathognomic cellular changes distinct from cellular necrosis