ER stress and the unfolded protein response in neurodegeneration
The clinical manifestation of neurodegenerative diseases is initiated by the selective
alteration in the functionality of distinct neuronal populations. The pathology of many …
alteration in the functionality of distinct neuronal populations. The pathology of many …
A lifetime of stress: ATF6 in development and homeostasis
RF Hillary, U FitzGerald - Journal of biomedical science, 2018 - Springer
Background Activating transcription factor 6 (ATF6) is an endoplasmic reticulum (ER)-
localised protein and member of the leucine zipper family of transcription factors. Best …
localised protein and member of the leucine zipper family of transcription factors. Best …
Evaluating the clinical validity of gene-disease associations: an evidence-based framework developed by the clinical genome resource
With advances in genomic sequencing technology, the number of reported gene-disease
relationships has rapidly expanded. However, the evidence supporting these claims varies …
relationships has rapidly expanded. However, the evidence supporting these claims varies …
Cellular stress signaling and the unfolded protein response in retinal degeneration: mechanisms and therapeutic implications
T McLaughlin, A Medina, J Perkins, M Yera… - Molecular …, 2022 - Springer
Background The retina, as part of the central nervous system (CNS) with limited capacity for
self-reparation and regeneration in mammals, is under cumulative environmental stress due …
self-reparation and regeneration in mammals, is under cumulative environmental stress due …
Small molecule strategies to harness the unfolded protein response: where do we go from here?
JMD Grandjean, RL Wiseman - Journal of Biological Chemistry, 2020 - ASBMB
The unfolded protein response (UPR) plays a central role in regulating endoplasmic
reticulum (ER) and global cellular physiology in response to pathologic ER stress. The UPR …
reticulum (ER) and global cellular physiology in response to pathologic ER stress. The UPR …
The endoplasmic reticulum: homeostasis and crosstalk in retinal health and disease
The endoplasmic reticulum (ER) is the largest intracellular organelle carrying out a broad
range of important cellular functions including protein biosynthesis, folding, and trafficking …
range of important cellular functions including protein biosynthesis, folding, and trafficking …
Pharmacologic ATF6 activating compounds are metabolically activated to selectively modify endoplasmic reticulum proteins
Pharmacologic arm-selective unfolded protein response (UPR) signaling pathway activation
is emerging as a promising strategy to ameliorate imbalances in endoplasmic reticulum (ER) …
is emerging as a promising strategy to ameliorate imbalances in endoplasmic reticulum (ER) …
The intertwined roles of oxidative stress and endoplasmic reticulum stress in glaucoma
DJ Hurley, C Normile, M Irnaten, C O'Brien - Antioxidants, 2022 - mdpi.com
Glaucoma is the leading cause of irreversible blindness worldwide, and the burden of the
disease continues to grow as the global population ages. Currently, the only treatment …
disease continues to grow as the global population ages. Currently, the only treatment …
Unravelling the genetics of inherited retinal dystrophies: Past, present and future
The identification of the genes underlying monogenic diseases has been of interest to
clinicians and scientists for many years. Using inherited retinal dystrophies as an example of …
clinicians and scientists for many years. Using inherited retinal dystrophies as an example of …
ER proteostasis control of neuronal physiology and synaptic function
G Martínez, S Khatiwada, M Costa-Mattioli… - Trends in neurosciences, 2018 - cell.com
Neuronal proteostasis is maintained by the dynamic integration of different processes that
regulate the synthesis, folding, quality control, and localization of proteins. The endoplasmic …
regulate the synthesis, folding, quality control, and localization of proteins. The endoplasmic …