The chemical chaperone sodium 4-phenylbutyrate improves the secretion of the protein C(A267T) mutant in CHO-K1 cells trough the GRASP55 pathway

Some inherited coagulation factor deficiencies are caused by intracellular retention or degradation of misfolded proteins, and chemical chaperones have been shown to reverse protein misfolding. The purpose of the present study was to investigate whether chemical chaperones may improve secretion of t...

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Autores principales: Chollet, Maria Eugenia, Skarpen, Ellen, Iversen, Nina, Sandset, Per Morten, Skretting, Grethe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2015
Materias:
Letter to the Editor
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4599753/
https://www.ncbi.nlm.nih.gov/pubmed/26457178
http://dx.doi.org/10.1186/s13578-015-0048-4
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author Chollet, Maria Eugenia
Skarpen, Ellen
Iversen, Nina
Sandset, Per Morten
Skretting, Grethe
author_facet Chollet, Maria Eugenia
Skarpen, Ellen
Iversen, Nina
Sandset, Per Morten
Skretting, Grethe
author_sort Chollet, Maria Eugenia
collection PubMed
description Some inherited coagulation factor deficiencies are caused by intracellular retention or degradation of misfolded proteins, and chemical chaperones have been shown to reverse protein misfolding. The purpose of the present study was to investigate whether chemical chaperones may improve secretion of the protein C(A267T) (PC(A267T)) mutant in a cellular model. Using stably transfected Chinese hamster ovary cells (CHO-K1) expressing PC(A267T) we demonstrate that sodium 4-phenylbutyrate (PBA) increased the secretion of PC(A267T) by approximately 4-fold in comparison with untreated cells, and that this secretion seemed to follow an unconventional pathway via the Golgi reassembly stacking protein (GRASP55).
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spelling pubmed-45997532015-10-10 The chemical chaperone sodium 4-phenylbutyrate improves the secretion of the protein C(A267T) mutant in CHO-K1 cells trough the GRASP55 pathway Chollet, Maria Eugenia Skarpen, Ellen Iversen, Nina Sandset, Per Morten Skretting, Grethe Cell Biosci Letter to the Editor Some inherited coagulation factor deficiencies are caused by intracellular retention or degradation of misfolded proteins, and chemical chaperones have been shown to reverse protein misfolding. The purpose of the present study was to investigate whether chemical chaperones may improve secretion of the protein C(A267T) (PC(A267T)) mutant in a cellular model. Using stably transfected Chinese hamster ovary cells (CHO-K1) expressing PC(A267T) we demonstrate that sodium 4-phenylbutyrate (PBA) increased the secretion of PC(A267T) by approximately 4-fold in comparison with untreated cells, and that this secretion seemed to follow an unconventional pathway via the Golgi reassembly stacking protein (GRASP55). BioMed Central 2015-10-09 /pmc/articles/PMC4599753/ /pubmed/26457178 http://dx.doi.org/10.1186/s13578-015-0048-4 Text en © Chollet et al. 2015 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Letter to the Editor
Chollet, Maria Eugenia
Skarpen, Ellen
Iversen, Nina
Sandset, Per Morten
Skretting, Grethe
The chemical chaperone sodium 4-phenylbutyrate improves the secretion of the protein C(A267T) mutant in CHO-K1 cells trough the GRASP55 pathway
title The chemical chaperone sodium 4-phenylbutyrate improves the secretion of the protein C(A267T) mutant in CHO-K1 cells trough the GRASP55 pathway
title_full The chemical chaperone sodium 4-phenylbutyrate improves the secretion of the protein C(A267T) mutant in CHO-K1 cells trough the GRASP55 pathway
title_fullStr The chemical chaperone sodium 4-phenylbutyrate improves the secretion of the protein C(A267T) mutant in CHO-K1 cells trough the GRASP55 pathway
title_full_unstemmed The chemical chaperone sodium 4-phenylbutyrate improves the secretion of the protein C(A267T) mutant in CHO-K1 cells trough the GRASP55 pathway
title_short The chemical chaperone sodium 4-phenylbutyrate improves the secretion of the protein C(A267T) mutant in CHO-K1 cells trough the GRASP55 pathway
title_sort chemical chaperone sodium 4-phenylbutyrate improves the secretion of the protein c(a267t) mutant in cho-k1 cells trough the grasp55 pathway
topic Letter to the Editor
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4599753/
https://www.ncbi.nlm.nih.gov/pubmed/26457178
http://dx.doi.org/10.1186/s13578-015-0048-4
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