The impact of the unfolded protein response on human disease

Blockade of XBP1 splicing by inhibition of IRE1  is a promising therapeutic option in multiple myeloma

(2012) N. Mimura et al.   BLOOD

The Role of Endoplasmic Reticulum in Hepatic Lipid Homeostasis and Stress Signaling

(2012) Suneng Fu et al.   Cell Metabolism

Atf6α-null mice are glucose intolerant due to pancreatic β-cell failure on a high-fat diet but partially resistant to diet-induced insulin resistance

(2012) Masahiro Usui et al.   METABOLISM-CLINICAL AND EXPERIMENTAL

The molecular basis for selective inhibition of unconventional mRNA splicing by an IRE1-binding small molecule

(2012) B. C. S. Cross et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Protein folding stress in neurodegenerative diseases: a glimpse into the ER

(2011) Soledad Matus et al.   CURRENT OPINION IN CELL BIOLOGY

Sodium Phenylbutyrate, a Drug With Known Capacity to Reduce Endoplasmic Reticulum Stress, Partially Alleviates Lipid-Induced Insulin Resistance and β-Cell Dysfunction in Humans

(2011) Changting Xiao et al.   DIABETES

The unfolded protein response transducer IRE1α prevents ER stress-induced hepatic steatosis

(2011) Kezhong Zhang et al.   EMBO JOURNAL

The unfolded protein response and its role in intestinal homeostasis and inflammation

(2011) Arthur Kaser et al.   EXPERIMENTAL CELL RESEARCH

Liver-specific loss of glucose-regulated protein 78 perturbs the unfolded protein response and exacerbates a spectrum of liver diseases in mice

(2011) Cheng Ji et al.   HEPATOLOGY

Endoplasmic reticulum-tethered transcription factor cAMP responsive element-binding protein, hepatocyte specific, regulates hepatic lipogenesis, fatty acid oxidation, and lipolysis upon metabolic stress in mice

(2011) Chunbin Zhang et al.   HEPATOLOGY

Roles of the 15-kDa Selenoprotein (Sep15) in Redox Homeostasis and Cataract Development Revealed by the Analysis of Sep 15 Knockout Mice

(2011) Marina V. Kasaikina et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders

(2011) Karen M. Doyle et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Membrane aberrancy and unfolded proteins activate the endoplasmic reticulum stress sensor Ire1 in different ways

(2011) Thanyarat Promlek et al.   MOLECULAR BIOLOGY OF THE CELL

Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy

(2011) Günter U Höglinger et al.   NATURE GENETICS

Regulation of glucose homeostasis through a XBP-1–FoxO1 interaction

(2011) Yingjiang Zhou et al.   NATURE MEDICINE

The transcription factor cyclic AMP–responsive element–binding protein H regulates triglyceride metabolism

(2011) Jung Hoon Lee et al.   NATURE MEDICINE

Endoplasmic Reticulum Ca2+ Handling in Excitable Cells in Health and Disease

(2011) G. E. Stutzmann et al.   PHARMACOLOGICAL REVIEWS

Dual and opposing roles of the unfolded protein response regulated by IRE1  and XBP1 in proinsulin processing and insulin secretion

(2011) A.-H. Lee et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Selective Inhibition of a Regulatory Subunit of Protein Phosphatase 1 Restores Proteostasis

(2011) P. Tsaytler et al.   SCIENCE

Unfolded Proteins Are Ire1-Activating Ligands That Directly Induce the Unfolded Protein Response

(2011) B. M. Gardner et al.   SCIENCE

CHOP and AP-1 cooperatively mediate PUMA expression during lipoapoptosis

(2010) Sophie C. Cazanave et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Identification of an Ire1alpha endonuclease specific inhibitor with cytotoxic activity against human multiple myeloma

(2010) I. Papandreou et al.   BLOOD

Endoplasmic Reticulum Stress and the Inflammatory Basis of Metabolic Disease

(2010) Gökhan S. Hotamisligil   CELL

Intracellular Ca2+ storage in health and disease: A dynamic equilibrium

(2010) Eva Sammels et al.   CELL CALCIUM

Regulation of Hepatic Gluconeogenesis by an ER-Bound Transcription Factor, CREBH

(2010) Min-Woo Lee et al.   Cell Metabolism

Tauroursodeoxycholic Acid May Improve Liver and Muscle but Not Adipose Tissue Insulin Sensitivity in Obese Men and Women

(2010) Marleen Kars et al.   DIABETES

Sustained production of spliced X-box binding protein 1 (XBP1) induces pancreatic beta cell dysfunction and apoptosis

(2010) F. Allagnat et al.   DIABETOLOGIA

The GCN2-ATF4 pathway is critical for tumour cell survival and proliferation in response to nutrient deprivation

(2010) Jiangbin Ye et al.   EMBO JOURNAL

Emerging therapies for the treatment of relapsed or refractory multiple myeloma

(2010) Meletios A. Dimopoulos et al.   EUROPEAN JOURNAL OF HAEMATOLOGY

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

(2010) Sonya G. Fonseca et al.   JOURNAL OF CLINICAL INVESTIGATION

A combination of functional polymorphisms in the CASP8, MMP1, IL10 and SEPS1 genes affects risk of non-small cell lung cancer

(2010) Kent Hart et al.   LUNG CANCER

Induction of Liver Steatosis and Lipid Droplet Formation in ATF6α-Knockout Mice Burdened with Pharmacological Endoplasmic Reticulum Stress

(2010) Keisuke Yamamoto et al.   MOLECULAR BIOLOGY OF THE CELL

Orm family proteins mediate sphingolipid homeostasis

(2010) David K. Breslow et al.   NATURE

Genome-wide association identifies multiple ulcerative colitis susceptibility loci

(2010) Dermot P B McGovern et al.   NATURE GENETICS

TLR activation of the transcription factor XBP1 regulates innate immune responses in macrophages

(2010) Fabio Martinon et al.   NATURE IMMUNOLOGY

The regulatory subunits of PI3K, p85α and p85β, interact with XBP-1 and increase its nuclear translocation

(2010) Sang Won Park et al.   NATURE MEDICINE

PERK promotes cancer cell proliferation and tumor growth by limiting oxidative DNA damage

(2010) E Bobrovnikova-Marjon et al.   ONCOGENE

Transcriptional Regulation of VEGF-A by the Unfolded Protein Response Pathway

(2010) Rajarshi Ghosh et al.   PLoS One

Targeted Mutation of the Mouse Grp94 Gene Disrupts Development and Perturbs Endoplasmic Reticulum Stress Signaling

(2010) Changhui Mao et al.   PLoS One

Inositol-requiring enzyme 1  is a key regulator of angiogenesis and invasion in malignant glioma

(2010) G. Auf et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

PERK in beta cell biology and insulin biogenesis

(2010) Douglas R. Cavener et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

The ATF6-Met[67]Val Substitution Is Associated With Increased Plasma Cholesterol Levels

(2009) Steven J.R. Meex et al.   ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY

IRE1α Kinase Activation Modes Control Alternate Endoribonuclease Outputs to Determine Divergent Cell Fates

(2009) Dan Han et al.   CELL

Translation Attenuation through eIF2α Phosphorylation Prevents Oxidative Stress and Maintains the Differentiated State in β Cells

(2009) Sung Hoon Back et al.   Cell Metabolism

The IRE1α-XBP1 Pathway of the Unfolded Protein Response Is Required for Adipogenesis

(2009) Haibo Sha et al.   Cell Metabolism

Reduced Apoptosis and Plaque Necrosis in Advanced Atherosclerotic Lesions of Apoe−/− and Ldlr−/− Mice Lacking CHOP

(2009) Edward Thorp et al.   Cell Metabolism

CHOP and the endoplasmic reticulum stress response in myelinating glia

(2009) Alexander Gow et al.   CURRENT OPINION IN NEUROBIOLOGY

Grp78 Heterozygosity Promotes Adaptive Unfolded Protein Response and Attenuates Diet-Induced Obesity and Insulin Resistance

(2009) R. Ye et al.   DIABETES

Targeting the endoplasmic reticulum-stress response as an anticancer strategy

(2009) Sandra J.M. Healy et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy

(2009) C. Hetz et al.   GENES & DEVELOPMENT

Alteration of the unfolded protein response modifies neurodegeneration in a mouse model of Marinesco–Sjögren syndrome

(2009) Lihong Zhao et al.   HUMAN MOLECULAR GENETICS

Elimination of the CDP-ethanolamine Pathway Disrupts Hepatic Lipid Homeostasis

(2009) Roberta Leonardi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Membrane expansion alleviates endoplasmic reticulum stress independently of the unfolded protein response

(2009) Sebastian Schuck et al.   JOURNAL OF CELL BIOLOGY

Regulated Ire1-dependent decay of messenger RNAs in mammalian cells

(2009) Julie Hollien et al.   JOURNAL OF CELL BIOLOGY

GRP78 expression inhibits insulin and ER stress–induced SREBP-1c activation and reduces hepatic steatosis in mice

(2009) Hélène L. Kammoun et al.   JOURNAL OF CLINICAL INVESTIGATION

The chemical chaperone 4-phenylbutyrate inhibits adipogenesis by modulating the unfolded protein response

(2009) Sana Basseri et al.   JOURNAL OF LIPID RESEARCH

Relationship between functional promoter polymorphism in the XBP1 gene (−116C/G) and atherosclerosis, ischemic stroke and hyperhomocysteinemia

(2009) Erkan Yilmaz et al.   MOLECULAR BIOLOGY REPORTS

The CREB coactivator CRTC2 links hepatic ER stress and fasting gluconeogenesis

(2009) Yiguo Wang et al.   NATURE

Adaptive suppression of the ATF4–CHOP branch of the unfolded protein response by toll-like receptor signalling

(2009) Connie W. Woo et al.   NATURE CELL BIOLOGY

In vitro reconstitution of ER-stress induced ATF6 transport in COPII vesicles

(2009) A. J. Schindler et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Function of IRE1 alpha in the placenta is essential for placental development and embryonic viability

(2009) T. Iwawaki et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ER Stress Controls Iron Metabolism Through Induction of Hepcidin

(2009) C. Vecchi et al.   SCIENCE

Attenuated BDNF-induced upregulation of GABAergic markers in neurons lacking Xbp1

(2008) Akiko Hayashi et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

XBP1 Links ER Stress to Intestinal Inflammation and Confers Genetic Risk for Human Inflammatory Bowel Disease

(2008) Arthur Kaser et al.   CELL

Real-Time Redox Measurements during Endoplasmic Reticulum Stress Reveal Interlinked Protein Folding Functions

(2008) Philip I. Merksamer et al.   CELL

ATF4-Mediated Induction of 4E-BP1 Contributes to Pancreatic β Cell Survival under Endoplasmic Reticulum Stress

(2008) Suguru Yamaguchi et al.   Cell Metabolism

UPR Pathways Combine to Prevent Hepatic Steatosis Caused by ER Stress-Mediated Suppression of Transcriptional Master Regulators

(2008) D. Thomas Rutkowski et al.   DEVELOPMENTAL CELL

CHOPT/C and C/T haplotypes contribute to early-onset type 2 diabetes in Italians

(2008) Claudia Gragnoli   JOURNAL OF CELLULAR PHYSIOLOGY

Inhibition of apolipoprotein B100 secretion by lipid-induced hepatic endoplasmic reticulum stress in rodents

(2008) Tsuguhito Ota et al.   JOURNAL OF CLINICAL INVESTIGATION

Chop deletion reduces oxidative stress, improves β cell function, and promotes cell survival in multiple mouse models of diabetes

(2008) Benbo Song et al.   JOURNAL OF CLINICAL INVESTIGATION

Membrane phospholipid synthesis and endoplasmic reticulum function

(2008) Paolo Fagone et al.   JOURNAL OF LIPID RESEARCH

Antioxidants reduce endoplasmic reticulum stress and improve protein secretion

(2008) J. D. Malhotra et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ATF6 -Rheb-mTOR signaling promotes survival of dormant tumor cells in vivo

(2008) D. M. Schewe et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Regulation of Hepatic Lipogenesis by the Transcription Factor XBP1

(2008) A.-H. Lee et al.   SCIENCE