Endoplasmic Reticulum Stress and Disease

Visual impairment cell non-autonomously dysregulates brain-wide proteostasis.

Drosophila and mouse models link loss of visual input to dysregulated stress responses of neurons and glia in the brain.

Non-canonical activation of IRE1a during Candida albicans infection enhances macrophage fungicidal activity

This study shows that Candida albicans activates IRE1 in macrophages through non-canonical mechanisms, enhancing fungicidal activity by promoting phagolysosomal fusion.

DELE1 oligomerization promotes integrated stress response activation

This study reports the cryo-EM structure of DELE1's C-terminal fragment, showing its oligomerization and role in activating the integrated stress response.

Monitoring integrated stress response in live Drosophila

The paper develops a fluorescent reporter system to monitor the integrated stress response in live Drosophila, revealing tissue-specific stress regulation during infections.

S-nitrosylation-mediated unfolded protein response maintains hematopoietic progenitors

This study identifies S-nitrosylation as a mechanism that maintains hematopoietic progenitor quiescence by inducing the unfolded protein response and blocking receptor function.

A homozygous p.(Arg371Ser) mutation in FICD de-regulates AMPylation of the human endoplasmic reticulum chaperone BiP causing infancy-onset diabetes and severe neurodevelopmental delay

FK506-binding protein 2 participates in proinsulin folding

This study identifies FKBP2 as a proline isomerase involved in proinsulin folding, showing its role in preventing misfolding and apoptosis in β-cells.

Control of mammalian brain aging by the unfolded protein response (UPR)

The paper shows that manipulating the UPR pathway can restore brain function and reduce aging-related deficits.

Protein synthesis inhibition and loss of homeostatic functions in astrocytes from an Alzheimer's disease mouse model: a role for ER-mitochondria interaction.

This study shows that in Alzheimer's disease astrocytes, protein synthesis is impaired through PERK-independent p-eIF2 activation, linked to altered ER-mitochondria interactions, affecting homeostatic functions.

A CREBZF/Zhangfei isoform activates CHOP during prolonged cellular stress

The paper identifies a CREBZF isoform, stZF, that activates CHOP during prolonged ER stress, promoting apoptosis and suggesting CREBZF's role in the Integrated Stress Response.

Hepatic Deletion of X-box Binding Protein 1 in Farnesoid X Receptor Null Mice Leads to Enhanced Liver Injury

This study investigates the role of the UPR in FXR null mice, finding that hepatic XBP1 activation provides protection against liver injury and tumor development.

Whole-body sorcin invalidation does not cause hypothalamic ER stress nor worsens obesity in C57BL/6 male mice fed a westernized diet.

This study investigates the role of sorcin in hypothalamic ER stress and obesity in mice, finding no significant effects on ER stress, body weight, or food intake.

Effect of Endoplasmic Reticulum Stress on Human Trophoblast Cells: Survival Triggering or Catastrophe Resulting in Death

This study investigates how Thapsigargin-induced ER stress affects apoptosis and autophagy in 2D and 3D human trophoblast cultures, revealing distinct survival and death pathways.

The Rab GTPase Ypt1p governs the activation of Unfolded Protein Response (UPR) in Saccharomyces cerevisiae by promoting the preferential nuclear degradation of pre-HAC1 mRNA

The paper shows Ypt1p regulates UPR by controlling pre-HAC1 mRNA decay through nuclear localization and interactions with the NNS/CTEXT complex.

Expression of Unfolded Protein Response Genes in Post-transplantation Liver Biopsies

The paper examines UPR gene expression in post-transplantation liver biopsies to understand its role in cholestatic liver injury, finding increased UPR pathway activity in hyperbilirubinemia cases.

Newly synthesized mRNA escapes translational repression during the acute phase of the mammalian unfolded protein response

The paper investigates how newly synthesized mRNA, specifically XBP1 mRNA, escapes translational repression during the acute phase of the UPR by accumulating long poly(A) tails, enhancing cell survival.

Autism-associated mutation in Hevin/Sparcl1 induces endoplasmic reticulum stress through structural instability

This study shows that an ASD-associated mutation in SPARCL1 impairs Hevin secretion by causing ER stress through structural instability.

Stress-induced protein disaggregation in the Endoplasmic Reticulum catalysed by BiP

The paper shows that ER stress activates a protein disaggregation system catalyzed by BiP, resolving aggregates in the ER using a novel live-cell monitoring method.

The dynamic effect of regulatory genetic variation on the in vivo ER stress transcriptional response

This study investigates how genetic variation affects the ER stress transcriptional response in different tissues and genotypes, finding tissue- and genotype-specific regulatory mechanisms.

Defective proinsulin handling modulates the MHC I bound peptidome and activates the inflammasome in β-cells

This study shows that impaired proinsulin handling in β-cells increases MHC-I bound peptides and activates inflammasomes, potentially contributing to autoimmune diabetes.

Molecular origin of somatostatin-positive neuron vulnerability

The paper identifies that somatostatin processing is a vulnerability factor in SST+ neurons under ER stress, linking chronic stress to behavioral deficits.

Anti-inflammatory effects of WFS1 in pancreatic beta-cells

This study shows that WFS1 deficiency in pancreatic beta-cells increases inflammation, leading to ER stress and cell death, and identifies potential therapeutic targets for Wolfram syndrome.

A Retrotranslocation Assay that Predicts Defective VCP/p97-Mediated Trafficking of a Retroviral Signal Peptide

The paper develops a retrotranslocation assay to identify critical amino acids in MMTV signal peptide for VCP/p97 interaction and trafficking, revealing functional defects in specific mutants.

A protein-trap allele reveals roles for Drosophila ATF4 in photoreceptor degeneration, oocyte maturation and wing development

This study uses a protein-trap allele to investigate Drosophila ATF4 (crc) functions in photoreceptor degeneration, oocyte maturation, and wing development.

Permissive epigenetic landscape facilitates distinct transcriptional signatures of activating transcription factor 6 in the liver

The paper identifies Atf6 target genes and shows that a permissive epigenetic landscape allows distinct transcriptional responses to Atf6 activation in liver stress conditions.

Genotype-phenotype correlation analysis and therapeutic development using a patient stem cell-derived disease model of Wolfram syndrome

This study uses a patient-derived stem cell model to show that a specific WFS1 variant causes mild Wolfram syndrome, which can be treated with chemical chaperones.

Creld2 function during unfolded protein response is essential for liver metabolism homeostasis

This study shows that Creld2 is essential for maintaining liver metabolism homeostasis by linking the unfolded protein response (UPR) to metabolic regulation, with sex-specific effects in fatty liver disease.

Precision genetic cellular models identify therapies protective against endoplasmic reticulum stress

The paper identifies compounds that correct aberrant molecular phenotypes in cellular models of glycosylation defects using a phenotypic screening approach.

Gastrodia-Uncaria Water Extract Inhibits Endoplasmic Reticulum Stress and Matrix Metalloproteinase in Protecting against Cerebral Ischemia

This study shows that Gastrodia-Uncaria water extract reduces endoplasmic reticulum stress and MMP activity, protecting against cerebral ischemia in rats.

Age-related decline of the unfolded protein response in the heart promotes protein misfolding and cardiac pathology

This study shows that the unfolded protein response declines with age in the heart, leading to protein misfolding and cardiac dysfunction.