Endoplasmic Reticulum Stress and Disease

Unfolded protein response is a critical mediator in Campylobacter jejuni pathogenesis and host defence

This study shows that Campylobacter jejuni activates the unfolded protein response (UPR) in human intestinal epithelial cells, which contributes to inflammation and pathogenesis.

Oligosaccharyltransferase is involved in targeting to ER-associated degradation

The paper identifies oligosaccharyltransferase (OST) subunits as key interactors in ER-associated degradation (ERAD), suggesting a role in late stages of quality control beyond N-glycosylation.

PERK inhibition rewires translational and CMGC protein kinase networks into an antiviral state

The paper shows that inhibiting PERK rewires host kinase networks to create an antiviral state during coronavirus infection.

Unique and conserved endoplasmic reticulum stress responses in neuroendocrine cells

The paper identifies unique and shared transcriptional responses to ER stress in various endocrine cell types using mRNA sequencing.

The cellular basis of mitochondrial stress signaling in the brain

The paper shows that mitochondrial metabolic activity determines ER stress signaling activation in neurons, conserved across Drosophila and human brains.

Medium-chain fatty acids mitigate endoplasmic reticulum stress in yeast cells

The paper shows that medium-chain fatty acids reduce ER stress in yeast by altering ER membrane properties and mitigating protein aggregation.

The integrated stress response promotes macrophage inflammation and migration in autoimmune diabetes

This study shows that inhibiting the integrated stress response (ISR) reduces macrophage inflammation and migration in a mouse model of type 1 diabetes.

FICD deficiency protects mice from hypertrophy-induced heart failure via BiP-mediated activation of the UPRER and ER-phagy

This study shows that FICD deficiency protects mice from heart failure by enhancing BiP-mediated UPRER and ER-phagy under stress.

Fbxo42 promotes the degradation of Ataxin-2 granules to trigger terminal Xbp1 signaling

This study shows Fbxo42 promotes degradation of Ataxin-2 granules, enabling Xbp1 translation and triggering cell death during terminal UPR activation.

Proteostasis and Unfolded Protein Response Dynamics in Human Neuron and Mouse Glia Co-culture Reveal Cell-Specific Aging Responses

This study uses DIA proteomics to analyze age-related changes in proteostasis and UPR dynamics in human neurons and mouse glia co-cultures, revealing cell-type-specific aging responses.

Abiotic and biotic stress activate the unfolded protein response in grapevine, with IRE1/bZIP60 signaling contributing to Botrytis cinerea resistance

This study shows that abiotic and biotic stresses activate the UPR in grapevine, with IRE1/bZIP60 signaling enhancing resistance to Botrytis cinerea.

Structural basis of an EMC:Spf1 insertase-dislocase complex in the eukaryotic endoplasmic reticulum

The paper presents a cryo-EM structure of the EMC:Spf1 supercomplex, showing how it integrates TMD insertion and dislocation activities for membrane protein quality control.

Secreted Protein Production is Improved by Controlling Endoplasmic Reticulum Stress Associated Protein Degradation

The paper shows that increasing proteasomal activity improves secreted protein production in ER-stressed cells, while autophagy inhibition also enhances secretion.

Polycystin-2 is cardioprotective against myocardial infarction by regulating the calcium-mediated ER stress response.

This paper shows that Polycystin-2 protects the heart from myocardial infarction by regulating calcium-mediated ER stress response in cardiomyocytes.

RNA triggers chronic stress during neuronal aging

This study identifies mitochondrial-derived double-stranded RNA as a source of RNA stress in aged neurons, which activates the ISR through PKR and contributes to neurodegeneration.

Functional integrity of the SEL1L-HRD1 complex is critical for ERAD and organismal viability

This paper shows that the SEL1L-HRD1 complex is essential for ERAD function and neonatal survival in mice, with specific mutations causing lethal defects.

The impact of ERUPR on mitochondrial integrity mediated by PDK4

This study shows PDK4 links ERUPR and mitoUPR, affecting mitochondrial integrity in aging and neurodegeneration.

Metabolite import via SLC33A1 enables ATF6 activation by endoplasmic reticulum stress

This study identifies SLC33A1 as a key regulator of ATF6 activation by ER stress, showing its role in metabolite transport and glycan processing.

Optogenetic Clustering of Human IRE1 Reveals Differential Regulation of Transcription and mRNA Splice Isoform Abundance by the UPR

This study uses optogenetics to isolate IRE1 activity in human cells, revealing its distinct transcriptional and splicing regulatory roles in the UPR.

Manganese suppresses tumor growth through hyper-activating IRE1α

This study identifies manganese as an IRE1 activator that suppresses tumor growth by inducing apoptosis through the unfolded protein response pathway.

eIF2B Activator Rescues Neonatal Lethality of an eIF2Bα Sugar Phosphate Binding Mutation Associated with Vanishing White Matter Disease

This paper shows that an eIF2B activator rescues neonatal lethality in a VWM mouse model but leads to age-related motor deficits and myelin loss.

Dissecting Branch-Specific Unfolded Protein Response Activation in Drug-Tolerant BRAF-Mutant Melanoma using Data-Independent Acquisition Mass Spectrometry

This study identifies branch-specific UPR proteomic targets in BRAF-mutant melanoma using DIA LC-MS, revealing differential XBP1s regulation linked to drug tolerance.

Structure and functional analysis of CDNF-BiP interaction reveal a role in endoplasmic reticulum proteostasis regulation

This study characterizes the CDNF-BiP interaction structurally and functionally, revealing CDNF's role as an antagonist of BiP nucleotide exchange and its neuroprotective effects.

Progressive Supranuclear Palsy PERK haplotype B selectively translates DLX1 promoting tau toxicity

This study identifies a PERK-B/DLX1 pathway as a novel driver of tau pathology in human PSP and flies, showing DLX1 promotes tau toxicity.

Protein folding stress transcriptionally reprograms muscle metabolism

This study shows that activating the UPR in muscle cells reprograms metabolism from carbohydrate to lipid metabolism through transcriptional changes.

Spatial Transcriptomics Analysis Uncovers ER stress in MANF-deficient Purkinje Cells Underlying Alcohol-induced Cerebellar Vulnerability in Mice

This study uses spatial transcriptomics to show that MANF deficiency in Purkinje cells increases ER stress and vulnerability to alcohol-induced neurodegeneration in a sex-dependent manner in mice.

Neuronal SEL1L-HRD1 ERAD regulates one-carbon metabolism and is essential for motor function and survival

This study shows that neuronal SEL1L-HRD1 ERAD is critical for one-carbon metabolism, motor function, and survival, with loss leading to severe neurological defects.

Functional rescue of a fatal ERAD mutation via alternative splicing

This study reports the discovery of using antisense oligonucleotides (ASOs) to rescue the biallelic SEL1L C141Y variant, offering a potential therapeutic strategy.

ATF4 activates a transcriptional program that chronically suppresses mTOR activity promoting neurodegeneration in Parkinson's disease models

This study identifies a mechanism by which chronic ATF4 activation suppresses mTORC1/2 activity, leading to PUMA-mediated neuronal death in Parkinson's disease models.

E2F deregulation compromises ER homeostasis by attenuating IRE1 activity

This study shows that E2F deregulation impairs ER homeostasis by reducing IRE1 activity, leading to DNA accumulation in Drosophila salivary glands.