Endoplasmic Reticulum Stress and Disease

This study identifies superdeath, a Drosophila metallopeptidase, as a modifier of ER stress-induced apoptosis by acting upstream of CDK5 in the JNK pathway, without affecting the unfolded protein response.

This paper develops a mouse model with an epitope-tagged BiP/GRP78 protein to study its interactome and role in ER protein misfolding in vivo.

The paper uses live-cell assays to study how mutations in rhodopsin, α-synuclein, and TDP-43 affect cell stress and signaling, revealing altered Ca2+ signaling and phosphodiesterase activity.

The paper identifies Bfr1 as a regulator of Pmt1 and Pmt2 translation, affecting unfolded protein O-mannosylation in yeast.

This study identifies a novel interaction between PERP and SERCA2b at ER-PM junctions under ER stress, linking autophagy to apoptosis.

The paper shows that ER stress sensors use a bidirectional mechanism to improve information about ER stress by integrating signals from unfolded proteins and chaperones.

This paper investigates the opposing yet coordinated roles of Nrf1 and Nrf2 in response to tunicamycin-induced ER stress, showing how they regulate gene expression and maintain cellular homeostasis.

The paper shows that multiple decay events regulate HAC1 mRNA splicing and the UPR in budding yeast by modulating degradation of splicing intermediates.

The paper investigates PERK's role in ER stress response by analyzing gene expression in PERK -/- MEFs, finding widespread PERK-dependent repression of ER-targeted genes.

This study shows that inhibiting IRE1α kinase/RNase reduces lung fibrosis in mice by blocking the terminal UPR, with two compounds demonstrating efficacy in preventing and reversing fibrosis.

The paper shows that a novel eIF2B activator prevents neurological defects in a mouse model of Vanishing White Matter disease by normalizing the transcriptome and proteome.

ISRIB ameliorates ISR deregulation and clinical signs in VWM mice.

The paper develops a model of the unfolded protein response in yeast and uses optimization to show chaperone-mediated sensors are more efficient than direct unfolded protein sensors.

The paper shows that ATF4-mediated transcriptional adaptation to ETC disturbance enhances progenitor cell proliferation in Drosophila.

This study identifies Baldspot/ELOVL6 as a modifier of the ER stress response and disease, showing its role in reducing cell death and degeneration in a Drosophila model.

The paper quantifies gene expression regulation at multiple layers in response to ER stress in cervical cancer cells over time, identifying shared and stress-specific regulatory events.

The paper identifies a new quality control pathway for integral membrane proteins involving RAC-Ssb that couples translation rate to the unfolded protein response.

This paper shows that FICD's adenylyltransferase activity induces ER stress and that its disulfide-bond formation modulates its activity in the context of a hyperactive mutant.