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Drosophila as a model for unfolded protein response research

Endoplasmic Reticulum (ER) is an organelle where most secretory and membrane proteins are synthesized, folded, and undergo further maturation. As numerous conditions can perturb such ER function, eukaryotic cells are equipped with responsive signaling pathways, widely referred to as the Unfolded Pro...

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Autor principal: Ryoo, Hyung Don
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Korean Society for Biochemistry and Molecular Biology 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4576952/
https://www.ncbi.nlm.nih.gov/pubmed/25999177
http://dx.doi.org/10.5483/BMBRep.2015.48.8.099
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author Ryoo, Hyung Don
author_facet Ryoo, Hyung Don
author_sort Ryoo, Hyung Don
collection PubMed
description Endoplasmic Reticulum (ER) is an organelle where most secretory and membrane proteins are synthesized, folded, and undergo further maturation. As numerous conditions can perturb such ER function, eukaryotic cells are equipped with responsive signaling pathways, widely referred to as the Unfolded Protein Response (UPR). Chronic conditions of ER stress that cannot be fully resolved by UPR, or conditions that impair UPR signaling itself, are associated with many metabolic and degenerative diseases. In recent years, Drosophila has been actively employed to study such connections between UPR and disease. Notably, the UPR pathways are largely conserved between Drosophila and humans, and the mediating genes are essential for development in both organisms, indicating their requirement to resolve inherent stress. By now, many Drosophila mutations are known to impose stress in the ER, and a number of these appear similar to those that underlie human diseases. In addition, studies have employed the strategy of overexpressing human mutations in Drosophila tissues to perform genetic modifier screens. The fact that the basic UPR pathways are conserved, together with the availability of many human disease models in this organism, makes Drosophila a powerful tool for studying human disease mechanisms. [BMB Reports 2015; 48(8): 445-453]
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spelling pubmed-45769522015-09-30 Drosophila as a model for unfolded protein response research Ryoo, Hyung Don BMB Rep Invited Mini Review Endoplasmic Reticulum (ER) is an organelle where most secretory and membrane proteins are synthesized, folded, and undergo further maturation. As numerous conditions can perturb such ER function, eukaryotic cells are equipped with responsive signaling pathways, widely referred to as the Unfolded Protein Response (UPR). Chronic conditions of ER stress that cannot be fully resolved by UPR, or conditions that impair UPR signaling itself, are associated with many metabolic and degenerative diseases. In recent years, Drosophila has been actively employed to study such connections between UPR and disease. Notably, the UPR pathways are largely conserved between Drosophila and humans, and the mediating genes are essential for development in both organisms, indicating their requirement to resolve inherent stress. By now, many Drosophila mutations are known to impose stress in the ER, and a number of these appear similar to those that underlie human diseases. In addition, studies have employed the strategy of overexpressing human mutations in Drosophila tissues to perform genetic modifier screens. The fact that the basic UPR pathways are conserved, together with the availability of many human disease models in this organism, makes Drosophila a powerful tool for studying human disease mechanisms. [BMB Reports 2015; 48(8): 445-453] Korean Society for Biochemistry and Molecular Biology 2015-08-31 /pmc/articles/PMC4576952/ /pubmed/25999177 http://dx.doi.org/10.5483/BMBRep.2015.48.8.099 Text en Copyright © 2015, Korean Society for Biochemistry and Molecular Biology http://creativecommons.org/licenses/by-nc/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Invited Mini Review
Ryoo, Hyung Don
Drosophila as a model for unfolded protein response research
title Drosophila as a model for unfolded protein response research
title_full Drosophila as a model for unfolded protein response research
title_fullStr Drosophila as a model for unfolded protein response research
title_full_unstemmed Drosophila as a model for unfolded protein response research
title_short Drosophila as a model for unfolded protein response research
title_sort drosophila as a model for unfolded protein response research
topic Invited Mini Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4576952/
https://www.ncbi.nlm.nih.gov/pubmed/25999177
http://dx.doi.org/10.5483/BMBRep.2015.48.8.099
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