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The impact of the unfolded protein response on human disease

The impact of the unfolded protein response on human disease

http://www.wikidata.org/entity/Q36062081

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A synthetic biology approach identifies the mammalian UPR RNA ligase RtcB Signal peptide peptidase functions in ERAD to cleave the unfolded protein response regulator XBP1u Glucocerebrosidase is shaking up the synucleinopathies Protective role of the endoplasmic reticulum protein mitsugumin23 against ultraviolet C-induced cell death Adaptive preconditioning in neurological diseases – therapeutic insights from proteostatic perturbations Wolfram Syndrome: Diagnosis, Management, and Treatment Role for the Unfolded Protein Response in Heart Disease and Cardiac Arrhythmias The Unfolded Protein Response and the Role of Protein Disulfide Isomerase in Neurodegeneration Unfolded Protein Response and Macroautophagy in Alzheimer's, Parkinson's and Prion Diseases Redox control of protein degradation The unfolded protein response in multiple sclerosis Experimental Evidence Shows Salubrinal, an eIF2α Dephosphorylation Inhibitor, Reduces Xenotoxicant-Induced Cellular Damage Endoplasmic reticulum stress and Nox-mediated reactive oxygen species signaling in the peripheral vasculature: potential role in hypertension The Role of Damage-Associated Molecular Patterns in Human Diseases: Part I - Promoting inflammation and immunity Endoplasmic reticulum stress and the related signaling networks in severe asthma Mechanisms of regulation of PFKFB expression in pancreatic and gastric cancer cells Integration of the unfolded protein and oxidative stress responses through SKN-1/Nrf Myc-driven overgrowth requires unfolded protein response-mediated induction of autophagy and antioxidant responses in Drosophila melanogaster Ablation of C/EBP homologous protein does not protect T17M RHO mice from retinal degeneration Inhibition of the Unfolded Protein Response Mechanism Prevents Cardiac Fibrosis Crosstalk between the unfolded protein response and NF-κB-mediated inflammation in the progression of chronic kidney disease Advances and New Concepts in Alcohol-Induced Organelle Stress, Unfolded Protein Responses and Organ Damage Stress responses from the endoplasmic reticulum in cancer Endoplasmic reticulum quality control and systemic amyloid disease: Impacting protein stability from the inside out Tumor progression and the different faces of the PERK kinase Mutations in the unfolded protein response regulator ATF6 cause the cone dysfunction disorder achromatopsia Functional characterization of an AQP0 missense mutation, R33C, that causes dominant congenital lens cataract, reveals impaired cell-to-cell adhesion Characterization of a Steroid Receptor Coactivator Small Molecule Stimulator that Overstimulates Cancer Cells and Leads to Cell Stress and Death Nitric oxide and superoxide mediate diesel particle effects in cytokine-treated mice and murine lung epithelial cells--implications for susceptibility to traffic-related air pollution Cell biology. Metabolic control of cell death Control of dopaminergic neuron survival by the unfolded protein response transcription factor XBP1 Brucella induces an unfolded protein response via TcpB that supports intracellular replication in macrophages Enhanced antitumor immunity contributes to the radio-sensitization of ehrlich ascites tumor by the glycolytic inhibitor 2-deoxy-D-glucose in mice The genetic architecture of the genome-wide transcriptional response to ER stress in the mouse Exploring the Conserved Role of MANF in the Unfolded Protein Response in Drosophila melanogaster Mutations in Nonessential eIF3k and eIF3l Genes Confer Lifespan Extension and Enhanced Resistance to ER Stress in Caenorhabditis elegans α-Synuclein is involved in manganese-induced ER stress via PERK signal pathway in organotypic brain slice cultures Phenotypic assays identify azoramide as a small-molecule modulator of the unfolded protein response with antidiabetic activity Defining cutaneous molecular pathobiology of arsenicals using phenylarsine oxide as a prototype COMSAT: Residue contact prediction of transmembrane proteins based on support vector machines and mixed integer linear programming.

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The impact of the unfolded protein response on human disease

http://www.wikidata.org/entity/Q36062081

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The impact of the unfolded protein response on human disease

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The impact of the unfolded protein response on human disease

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Randal J Kaufman

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Shiyu Wang

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P2860

A gene encoding a transmembrane protein is mutated in patients with diabetes mellitus and optic atrophy (Wolfram syndrome)

XBP1 links ER stress to intestinal inflammation and confers genetic risk for human inflammatory bowel disease

LHS1 and SIL1 provide a lumenal function that is essential for protein translocation into the endoplasmic reticulum

Plasma cell differentiation requires the transcription factor XBP-1

XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor

Dimerization and release of molecular chaperone inhibition facilitate activation of eukaryotic initiation factor-2 kinase in response to endoplasmic reticulum stress

TRB3, a novel ER stress-inducible gene, is induced via ATF4-CHOP pathway and is involved in cell death

ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats

Wolfram syndrome 1 gene negatively regulates ER stress signaling in rodent and human cells

ORMDL proteins are a conserved new family of endoplasmic reticulum membrane proteins

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10.1083/JCB.201110131

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