Results 121 to 130 of about 28,738 (248)

Chemical chaperone treatment reduces intracellular accumulation of mutant collagen IV and ameliorates the cellular phenotype of a COL4A2 mutation that causes haemorrhagic stroke [PDF]

, 2013
Haemorrhagic stroke accounts for approximately 20% of stroke cases and porencephaly is a clinical consequence of perinatal cerebral haemorrhaging. Here we report the identification of a novel dominant G702D mutation in the collagen domain of COL4A2 ...
Aislynn Taggart, Bateman, Brazel, Catheline Vilain, de Clerck, Engin, Favor, Geesin, Gould, Gould, Gould, Hostikka, Ishida, Jeanne, Kalluri, Karl E. Kadler, Khoshnoodi, Lydia S. Murray, Marc Abramowicz, Muthukumaran, Nicole Van Regemorter, Ozcan, Plaisier, Plaisier, Poschl, Ron, Taylor, Tom Van Agtmael, Vahedi, Van Agtmael, Van Agtmael, Van Agtmael, Verbeek, Vilain, Woo, Yinhui Lu   +35 more
core   +1 more source

The effect of salubrinal on the endoplasmic reticulum stress pathway in heat‐stressed spermatogonial cells in vitro

FEBS Open Bio, Volume 16, Issue 2, Page 340-351, February 2026.
This study investigates the protective role of salubrinal against heat‐induced endoplasmic reticulum (ER) stress in mouse spermatogenic cells (GC1 and GC2). By modulating the ER stress pathway, salubrinal alleviates cellular stress and supports spermatogenic cell survival, suggesting its potential as a therapeutic candidate for heat‐related infertility.
Suna Karadeniz Saygili, Mustafa Oztatlici, Mahmut Kemal Ozbilgin   +2 more
wiley   +1 more source

ATF6-Mediated Unfolded Protein Response Facilitates Adeno-associated Virus 2 (AAV2) Transduction by Releasing the Suppression of the AAV Receptor on Endoplasmic Reticulum Stress

, 2021
Mengtian Cui, Qingfang Zhao, Jing Wang, Yang Si, Shan Cheng, Wei Ding   +5 more
openalex   +2 more sources

Hypercapnia Effects on Lung Function

Comprehensive Physiology, Volume 16, Issue 1, February 2026.
CO2 is a signaling molecule that disrupts alveolar fluid clearance and ion transport, impairs alveolar repair, and promotes mitochondrial and endoplasmic reticulum dysfunction. It suppresses innate immunity, host defense, and inflammation, while increasing airway contractility.
Seungseo Choi, Laura A. Dada, Jacob I. Sznajder   +2 more
wiley   +1 more source

α-Synuclein-mediated inhibition of ATF6 processing into COPII vesicles disrupts UPR signaling in Parkinson's disease

Neurobiology of Disease, 2015
The unfolded protein response (UPR) monitors the folding environment within the endoplasmic reticulum (ER). Accumulation of misfolded proteins within the ER activates the UPR resulting in the execution of adaptive or non-adaptive signaling pathways.
Joel J. Credle, Patrick A. Forcelli, Michael Delannoy, Adam W. Oaks, Eva Permaul, Deborah L. Berry, Valeriy Duka, Jonathan Wills, Anita Sidhu   +8 more
doaj   +1 more source

Proteostasis and ageing: insights from long-lived mutant mice [PDF]

, 2017
The global increase in life expectancy is creating significant medical, social and economic challenges to current and future generations. Consequently, there is a need to identify the fundamental mechanisms underlying the ageing process.
Page, Melissa M., Sands, William A., Selman, Colin   +2 more
core   +1 more source

Is Ferroptosis the Mechanistic Bridge Connecting Iron Dysregulation to Muscle Wasting and Functional Decline in Aging?

Aging Cell, Volume 25, Issue 2, February 2026.
This work proposes ferroptosis as a mechanistic driver of age‐related muscle dysfunction, whereby iron dyshomeostasis and impaired antioxidant capacity promote lipid peroxidation, and eventually trigger ferroptosis, leading to muscle wasting, and ultimately contributing to weakness and functional decline with aging.
Rola S. Zeidan, Simon Reinhard, Anna Picca, Emanuele Marzetti, Christiaan Leeuwenburgh, James F. Collins, Stephen D. Anton   +6 more
wiley   +1 more source

Pancreatic adaptive responses in alcohol abuse: Role of the unfolded protein response. [PDF]

, 2015
The majority of those who drink excessive amounts of alcohol do not develop pancreatic disease. One overarching hypothesis is that alcohol abuse requires additional risk factors, either environmental or genetic, for disease to occur.
Lugea, Aurelia, Pandol, Stephen J, Waldron, Richard T   +2 more
core   +1 more source

Supplementary Figures and Tables from ATF6-Mediated Signaling Contributes to PARP Inhibitor Resistance in Ovarian Cancer

, 2023
Alexandra McMellen, Tomomi M. Yamamoto, Lubna Qamar, Brooke E. Sanders, Lily L. Nguyen, Daniela Ortiz Chavez, Jaidev Bapat, Amber Berning, Zain Aryanpour, Joshua Johnson, Kian Behbakht, Elmar Nurmemmedov, Edward B. Chuong, Benjamin G. Bitler   +13 more
openalex   +1 more source

Mutations in unfolded protein response regulator ATF6 cause hearing and vision loss syndrome

The Journal of Clinical Investigation
Activating transcription factor 6 (ATF6) is a key regulator of the unfolded protein response (UPR) and is important for ER function and protein homeostasis in metazoan cells.
Eun-Jin Lee, Kyle Kim, Monica Sophia Diaz-Aguilar, Hyejung Min, Eduardo Chavez, Korina J. Steinbergs, Lance A. Safarta, Guirong Zhang, Allen F. Ryan, Jonathan H. Lin   +9 more
doaj   +1 more source