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Keywords: Unfolded protein response
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Journal Articles

New Drosophila models to uncover the intrinsic and extrinsic factors that mediate the toxicity of the human prion protein

In collection:
Drosophila as a Disease Model
Ryan R. Myers, Jonatan Sanchez-Garcia, Daniel C. Leving, Richard G. Melvin, Pedro Fernandez-Funez
Journal: Disease Models & Mechanisms
Dis Model Mech (2022) 15 (4): dmm049184.
https://doi.org/10.1242/dmm.049184
Published: 3 May 2022
... demonstrate the heightened toxicity of human PrP compared to hamster and mouse PrP. As proof-of-concept for the utility of the new human PrP model, we identified intrinsic and extrinsic factors modulating its toxicity. Accumulation of misfolded PrP in the ER triggers the unfolded protein response (UPR) ( Hetz...
View article titled, New Drosophila models to uncover the intrinsic and extrinsic factors that mediate the toxicity of the human prion protein
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Includes: Supplementary data
Journal Articles

Propensity to endoplasmic reticulum stress in deer mouse fibroblasts predicts skin inflammation and body weight gain

In collection:
Metabolic Disorders , Tools and Resources for Mouse Studies
Youwen Zhang, Chang-uk Lim, Vitali Sikirzhytski, Asieh Naderi, Ioulia Chatzistamou, Hippokratis Kiaris
Journal: Disease Models & Mechanisms
Dis Model Mech (2021) 14 (10): dmm049113.
https://doi.org/10.1242/dmm.049113
Published: 18 October 2021
...Youwen Zhang; Chang-uk Lim; Vitali Sikirzhytski; Asieh Naderi; Ioulia Chatzistamou; Hippokratis Kiaris ABSTRACT The unfolded protein response (UPR) is involved in the pathogenesis of metabolic disorders, yet whether variations in the UPR among individuals influence the propensity for metabolic...
View article titled, Propensity to endoplasmic reticulum stress in deer mouse fibroblasts predicts skin inflammation and body weight gain
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Includes: Supplementary data
Journal Articles

Establishment and validation of an endoplasmic reticulum stress reporter to monitor zebrafish ATF6 activity in development and disease

In collection:
Neurodegenerative Disorders , Zebrafish as a Disease Model
Eric M. Clark, Hannah J. T. Nonarath, Jonathan R. Bostrom, Brian A. Link
Journal: Disease Models & Mechanisms
Dis Model Mech (2020) 13 (1): dmm041426.
https://doi.org/10.1242/dmm.041426
Published: 28 January 2020
... generated to specifically report the activity of ATF6, representing a major branch of the endoplasmic reticulum stress pathway with functions in development and disease. ATF6 Endoplasmic reticulum stress Unfolded protein response Zebrafish Neurodegeneration National Institutes of Health...
View article titled, Establishment and validation of an endoplasmic reticulum stress reporter to monitor zebrafish ATF6 activity in development and disease
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Includes: Supplementary data
Journal Articles

SILAC-based quantitative proteomics using mass spectrometry quantifies endoplasmic reticulum stress in whole HeLa cells

Daniel N. Itzhak, Francesca Sacco, Nagarjuna Nagaraj, Stefka Tyanova, Matthias Mann, Marta Murgia
Journal: Disease Models & Mechanisms
Dis Model Mech (2019) 12 (11): dmm040741.
https://doi.org/10.1242/dmm.040741
Published: 11 November 2019
...Daniel N. Itzhak; Francesca Sacco; Nagarjuna Nagaraj; Stefka Tyanova; Matthias Mann; Marta Murgia ABSTRACT The unfolded protein response (UPR) involves extensive proteome remodeling in many cellular compartments. To date, a comprehensive analysis of the UPR has not been possible because...
View article titled, SILAC-based quantitative proteomics using mass spectrometry quantifies endoplasmic reticulum stress in whole HeLa cells
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Includes: Supplementary data
Journal Articles

Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chaperone 4-phenylbutyrate

In collection:
Rare Disease Translational Research
Roberta Besio, Nadia Garibaldi, Laura Leoni, Lina Cipolla, Simone Sabbioneda, Marco Biggiogera, Monica Mottes, Mona Aglan, Ghada A. Otaify, Samia A. Temtamy, Antonio Rossi, Antonella Forlino
Journal: Disease Models & Mechanisms
Dis Model Mech (2019) 12 (6): dmm038521.
https://doi.org/10.1242/dmm.038521
Published: 20 June 2019
... with the presence of protein aggregates, activation of the PERK branch of the unfolded protein response and apoptotic death. The administration of 4-phenylbutyrate (4-PBA) alleviates cellular stress by restoring ER cisternae size, and normalizing the phosphorylated PERK (p-PERK):PERK ratio and the expression...
View article titled, Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chaperone 4-phenylbutyrate
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Includes: Supplementary data
Journal Articles

Molecularly defined unfolded protein response subclasses have distinct correlations with fatty liver disease in zebrafish

Ana M. Vacaru, Antonio Fabio Di Narzo, Deanna L. Howarth, Orkhontuya Tsedensodnom, Dru Imrie, Ayca Cinaroglu, Salma Amin, Ke Hao, Kirsten C. Sadler
Journal: Disease Models & Mechanisms
Dis Model Mech (2014) 7 (7): 823–835.
https://doi.org/10.1242/dmm.014472
Published: 1 July 2014
...Ana M. Vacaru; Antonio Fabio Di Narzo; Deanna L. Howarth; Orkhontuya Tsedensodnom; Dru Imrie; Ayca Cinaroglu; Salma Amin; Ke Hao; Kirsten C. Sadler The unfolded protein response (UPR) is a complex network of sensors and target genes that ensure efficient folding of secretory proteins...
View article titled, Molecularly defined <span class="search-highlight">unfolded</span> <span class="search-highlight">protein</span> <span class="search-highlight">response</span> subclasses have distinct correlations with fatty liver disease in zebrafish
Open the PDF for Molecularly defined <span class="search-highlight">unfolded</span> <span class="search-highlight">protein</span> <span class="search-highlight">response</span> subclasses have distinct correlations with fatty liver disease in zebrafish in another window
Includes: Supplementary data