Endoplasmic Reticulum Stress and Disease

The paper uses a CRISPR/Cas9 screen to identify calreticulin as a repressor of ATF6α signaling in the unfolded protein response.

The paper identifies a mechanism by which eIF2γ binding to PPP1R15A enhances catalytic efficiency of the holophosphatase that terminates the integrated stress response.

This study shows that BAX and BAK deficiency increases ER stress threshold for XBP1 splicing and reduces RIDD substrate degradation and IRE1 phosphorylation.

The paper investigates how IL-1 induces senescence in adult neural stem cells via the ATF4 pathway following traumatic brain injury.

The paper shows that glutamate receptor activation causes ER Ca2+ depletion, leading to secretion of ER resident proteins and disrupting proteostasis during excitotoxicity.

The paper compares how PDI and ERp46 recognize client proteins during oxidative folding, showing distinct mechanisms that influence disulfide bond formation and protein quality control.

This study investigates the effects of Salubrinal and ISRIB on eIF2 phosphorylation in TBI-induced traumatic optic neuropathy, finding that both drugs impair visual function in control animals and show limited benefit in injured mice.

This study shows that Nwd1 knockout mice develop NASH-like liver pathology due to SERCA2 dysfunction and ER stress.

This study shows that depletion of DNA primases in C. elegans activates the endoplasmic reticulum unfolded protein response (UPR-ER), which is required to alleviate replication stress.

This study investigates how ER stress signaling contributes to therapy resistance in colorectal cancer by analyzing genomic alterations and testing therapeutic responses in cell lines and organoids.

This study identifies CDC5L as a transcriptional repressor that prevents spontaneous stress granule formation by suppressing PERK-eIF2 signaling.

This study investigates how a WFS1 splice site mutation affects β cell apoptosis in Wolfram syndrome by analyzing alternative splicing and functional consequences in patient-derived iPSCs.

The paper identifies histone methylation, particularly via the Set1/COMPASS complex, as a key modifier of ER stress preconditioning in Drosophila.

The paper identifies YIPF5 and GOT1A/B as components of ER export and shows ATF6 has a transcription-independent role in ER export inhibition by FLI-06.

The paper shows that ERLAD compensates for ERAD dysfunction by delivering misfolded proteins to lysosomes when ERAD is inhibited.

This study shows that increased IRE1 activity in a mouse model of EAE reduces inflammation and improves motor function, suggesting context-dependent protective effects of ER stress.

This study shows that Slt2 in yeast enhances HAC1 mRNA splicing and translation during ER stress, and human ERK1/2 can substitute for Slt2 in UPR.

DNL343 reduces neurodegeneration in mouse models by inhibiting the integrated stress response and normalizing dysregulated biomarkers, with potential clinical relevance for vanishing white matter disease.

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

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

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

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

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

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

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

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.

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.

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.

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.