The role of the ER stress-response protein PERK in rhodopsin retinitis pigmentosa

NLRP3 inflammasome activation drives bystander cone photoreceptor cell death in a P23H rhodopsin model of retinal degeneration

(2016) Ishaq A. Viringipurampeer et al.   HUMAN MOLECULAR GENETICS

Limited ATF4 Expression in Degenerating Retinas with Ongoing ER Stress Promotes Photoreceptor Survival in a Mouse Model of Autosomal Dominant Retinitis Pigmentosa

(2016) Yogesh Bhootada et al.   PLoS One

Genotypic and Phenotypic Characterization of P23H Line 1 Rat Model

(2015) Elise Orhan et al.   PLoS One

The co-chaperone and reductase ERdj5 facilitates rod opsin biogenesis and quality control

(2014) Dimitra Athanasiou et al.   HUMAN MOLECULAR GENETICS

Robust Endoplasmic Reticulum-Associated Degradation of Rhodopsin Precedes Retinal Degeneration

(2014) Wei-Chieh Chiang et al.   MOLECULAR NEUROBIOLOGY

Ablation of the Proapoptotic Genes Chop or Ask1 Does Not Prevent or Delay Loss of Visual Function in a P23H Transgenic Mouse Model of Retinitis Pigmentosa

(2014) Adeseye Adekeye et al.   PLoS One

The heat-shock response co-inducer arimoclomol protects against retinal degeneration in rhodopsin retinitis pigmentosa

(2014) D A Parfitt et al.   Cell Death & Disease

The cell stress machinery and retinal degeneration

(2013) Dimitra Athanasiou et al.   FEBS LETTERS

Hsp90 inhibition protects against inherited retinal degeneration

(2013) Mònica Aguilà et al.   HUMAN MOLECULAR GENETICS

P23H opsin knock-in mice reveal a novel step in retinal rod disc morphogenesis

(2013) S. Sakami et al.   HUMAN MOLECULAR GENETICS

Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models

(2013) Hyung-Jun Kim et al.   NATURE GENETICS

Suppression of eIF2α kinases alleviates Alzheimer's disease–related plasticity and memory deficits

(2013) Tao Ma et al.   NATURE NEUROSCIENCE

Ablation of C/EBP Homologous Protein Does Not Protect T17M RHO Mice from Retinal Degeneration

(2013) Sonali Nashine et al.   PLoS One

Oral Treatment Targeting the Unfolded Protein Response Prevents Neurodegeneration and Clinical Disease in Prion-Infected Mice

(2013) J. A. Moreno et al.   Science Translational Medicine

Pharmacological Modulation of the Retinal Unfolded Protein Response in Bardet-Biedl Syndrome Reduces Apoptosis and Preserves Light Detection Ability

(2012) Anais Mockel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Discovery of 7-Methyl-5-(1-{[3-(trifluoromethyl)phenyl]acetyl}-2,3-dihydro-1H-indol-5-yl)-7H-pyrrolo[2,3-d]pyrimidin-4-amine (GSK2606414), a Potent and Selective First-in-Class Inhibitor of Protein Kinase R (PKR)-like Endoplasmic Reticulum Kinase (PERK)

(2012) Jeffrey M. Axten et al.   JOURNAL OF MEDICINAL CHEMISTRY

BiP prevents rod opsin aggregation

(2012) Dimitra Athanasiou et al.   MOLECULAR BIOLOGY OF THE CELL

Sustained translational repression by eIF2α-P mediates prion neurodegeneration

(2012) Julie A. Moreno et al.   NATURE

ER Stress in Retinal Degeneration in S334ter Rho Rats

(2012) Vishal M. Shinde et al.   PLoS One

Probing Mechanisms of Photoreceptor Degeneration in a New Mouse Model of the Common Form of Autosomal Dominant Retinitis Pigmentosa due to P23H Opsin Mutations

(2011) Sanae Sakami et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Calpain and PARP Activation during Photoreceptor Cell Death in P23H and S334ter Rhodopsin Mutant Rats

(2011) Jasvir Kaur et al.   PLoS One

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

Clearance of RhodopsinP23H aggregates requires the ERAD effector VCP

(2010) Ana Griciuc et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

The unfolded protein response in familial amyotrophic lateral sclerosis

(2010) L. Wang et al.   HUMAN MOLECULAR GENETICS

The UPR and cell fate at a glance

(2010) P. I. Merksamer et al.   JOURNAL OF CELL SCIENCE

Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78

(2010) M. S. Gorbatyuk et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ER stress protects from retinal degeneration

(2009) César S Mendes et al.   EMBO JOURNAL

A dual role for EDEM1 in the processing of rod opsin

(2009) Maria Kosmaoglou et al.   JOURNAL OF CELL SCIENCE

Suppression of retinal degeneration in Drosophila by stimulation of ER-associated degradation

(2009) M.-J. Kang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The relationship of photoreceptor degeneration to retinal vascular development and loss in mutant rhodopsin transgenic and RCS rats

(2008) Mark E. Pennesi et al.   EXPERIMENTAL EYE RESEARCH

Pharmacological manipulation of gain-of-function and dominant-negative mechanisms in rhodopsin retinitis pigmentosa

(2008) Hugo F. Mendes et al.   HUMAN MOLECULAR GENETICS