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Related Concept Videos

Regulation of the Unfolded Protein Response01:31

Regulation of the Unfolded Protein Response

Inositol-requiring kinase one or IRE1 is the most conserved eukaryotic unfolded protein response (UPR) receptor. It is a type I transmembrane protein kinase receptor with a distinctive site-specific RNase activity. As the binding mechanics of the misfolded proteins with the N-terminal domain of IRE-1 are unclear, three binding models — direct, indirect, and allosteric -- are proposed for receptor activation. Nevertheless, it is known that once a misfolded protein associates with IRE1, it...
Export of Misfolded Proteins out of the ER01:32

Export of Misfolded Proteins out of the ER

After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...
The Unfolded Protein Response01:37

The Unfolded Protein Response

The ER is the hub of protein synthesis in a cell. It has robust systems to quality control protein folding and also for degradation of terminally misfolded proteins. Under normal conditions, a small proportion of misfolded proteins that cannot be salvaged need to be transported to the cytoplasm by the ER-associated degradation or ERAD pathways. However, if the ERAD cannot handle the misfolded proteins, the cell activates the unfolded protein response or UPR to adjust the protein folding...

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Related Experiment Video

Updated: May 8, 2026

A Protocol to Evaluate and Quantify Retinal Pigmented Epithelium Pathologies in Mouse Models of Age-Related Macular Degeneration
09:24

A Protocol to Evaluate and Quantify Retinal Pigmented Epithelium Pathologies in Mouse Models of Age-Related Macular Degeneration

Published on: March 10, 2023

ERK1/2 pathway is activated in degenerated Rpe65-deficient mice.

S Métrailler1, M Emery, D F Schorderet

  • 1IRO, Institute for Research in Ophthalmology, 1950 Sion, Switzerland.

Experimental Eye Research
|September 10, 2013
PubMed
Summary
This summary is machine-generated.

In Rpe65(-/-) mice, ERK1/2 activation and GFAP increase indicate atypical Müller cell gliosis during photoreceptor degeneration, without cell proliferation.

Keywords:
ERK1/2Leber congenital amaurosisatypical Müller gliosisretinal degeneration

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Last Updated: May 8, 2026

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Nitroreductase/Metronidazole-Mediated Ablation and a MATLAB Platform (RpEGEN) for Studying Regeneration of the Zebrafish Retinal Pigment Epithelium
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Nitroreductase/Metronidazole-Mediated Ablation and a MATLAB Platform (RpEGEN) for Studying Regeneration of the Zebrafish Retinal Pigment Epithelium

Published on: March 2, 2022

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Ophthalmology

Background:

  • Mitogen-activated protein kinase (MAPK) pathways, including ERK1/2, are involved in cellular stress and degenerative processes.
  • Retinal cell death and Müller cell gliosis are key features in various retinal degenerative diseases.

Purpose of the Study:

  • To investigate the activation of extracellular signal-regulated kinase 1/2 (ERK1/2) and its role in Müller cell gliosis during photoreceptor cell death in Rpe65(-/-) mice.
  • To determine if ERK1/2 activation correlates with glial activation markers and retinal degeneration.

Main Methods:

  • Assessed ERK1/2 mRNA, protein levels, and phosphorylation in Rpe65(-/-) and wild-type mice at different ages (2, 4, 6 months).
  • Evaluated co-localization of phosphorylated ERK1/2 (pERK1/2) with glial fibrillary acidic protein (GFAP) and cFOS.
  • Monitored Müller cell proliferation and degeneration progression.

Main Results:

  • ERK1/2 phosphorylation significantly increased in Rpe65(-/-) mice at 4 and 6 months, correlating with retinal cell death and increased GFAP protein.
  • pERK1/2 and GFAP were localized in the ganglion cell layer, with concomitant cFOS accumulation.
  • No Müller cell proliferation was observed, and ERK1/2 activation was absent in mice without degeneration.

Conclusions:

  • The findings suggest an atypical form of Müller cell gliosis, characterized by ERK1/2 and GFAP activation without proliferation, in Rpe65(-/-) mice during slow photoreceptor degeneration.
  • Further research is needed to elucidate the role of this atypical gliosis in the pathogenesis of Leber congenital amaurosis model.