Orchestrating the unfolded protein response in health and disease

Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism

1998 • 5,063 citations

XBP1 mRNA Is Induced by ATF6 and Spliced by IRE1 in Response to ER Stress to Produce a Highly Active Transcription Factor

2001 • 3,799 citations

Caspase-12 mediates endoplasmic-reticulum-specific apoptosis and cytotoxicity by amyloid-β

2000 • 3,241 citations

Regulated Translation Initiation Controls Stress-Induced Gene Expression in Mammalian Cells

2000 • 3,071 citations

Coupling of Stress in the ER to Activation of JNK Protein Kinases by Transmembrane Protein Kinase IRE1

2000 • 2,831 citations

IRE1 couples endoplasmic reticulum load to secretory capacity by processing the XBP-1 mRNA

2002 • 2,702 citations

Stress signaling from the lumen of the endoplasmic reticulum: coordination of gene transcriptional and translational controls

1999 • 2,237 citations

Impairment of the Ubiquitin-Proteasome System by Protein Aggregation

2001 • 2,167 citations

Mammalian Transcription Factor ATF6 Is Synthesized as a Transmembrane Protein and Activated by Proteolysis in Response to Endoplasmic Reticulum Stress

1999 • 1,954 citations

Functional and Genomic Analyses Reveal an Essential Coordination between the Unfolded Protein Response and ER-Associated Degradation

2000 • 1,939 citations

ER Stress Induces Cleavage of Membrane-Bound ATF6 by the Same Proteases that Process SREBPs

2000 • 1,764 citations

ER Stress Regulation of ATF6 Localization by Dissociation of BiP/GRP78 Binding and Unmasking of Golgi Localization Signals

2002 • 1,368 citations

Translational Control Is Required for the Unfolded Protein Response and In Vivo Glucose Homeostasis

2001 • 1,329 citations

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

2002 • 1,296 citations

Plasma cell differentiation requires the transcription factor XBP-1

2001 • 1,256 citations

Identification of the cis-Acting Endoplasmic Reticulum Stress Response Element Responsible for Transcriptional Induction of Mammalian Glucose-regulated Proteins

1998 • 1,216 citations

Diabetes Mellitus and Exocrine Pancreatic Dysfunction in Perk−/− Mice Reveals a Role for Translational Control in Secretory Cell Survival

2001 • 1,204 citations

Cross-Talk between Two Cysteine Protease Families

2000 • 1,158 citations

An Unfolded Putative Transmembrane Polypeptide, which Can Lead to Endoplasmic Reticulum Stress, Is a Substrate of Parkin

2001 • 1,048 citations

IRE1-mediated unconventional mRNA splicing and S2P-mediated ATF6 cleavage merge to regulate XBP1 in signaling the unfolded protein response

2002 • 1,031 citations

The glucose-regulated proteins: stress induction and clinical applications

2001 • 1,013 citations

ATF6 Activated by Proteolysis Binds in the Presence of NF-Y (CBF) Directly to thecis-Acting Element Responsible for the Mammalian Unfolded Protein Response

2000 • 921 citations

Tripartite Management of Unfolded Proteins in the Endoplasmic Reticulum

2000 • 917 citations

A stress response pathway from the endoplasmic reticulum to the nucleus requires a novel bifunctional protein kinase/endoribonuclease (Ire1p) in mammalian cells

1998 • 888 citations

EIF2AK3, encoding translation initiation factor 2-α kinase 3, is mutated in patients with Wolcott-Rallison syndrome

2000 • 800 citations

Cloning of mammalian Ire1 reveals diversity in the ER stress responses

1998 • 782 citations

Intracellular signaling from the endoplasmic reticulum to the nucleus: the unfolded protein response in yeast and mammals

2001 • 772 citations

Parkin Suppresses Unfolded Protein Stress-induced Cell Death through Its E3 Ubiquitin-protein Ligase Activity

2000 • 757 citations

Targeted disruption of the Chop gene delays endoplasmic reticulum stress–mediated diabetes

2002 • 724 citations

Homocysteine-induced endoplasmic reticulum stress causes dysregulation of the cholesterol and triglyceride biosynthetic pathways

2001 • 696 citations

Complementary Signaling Pathways Regulate the Unfolded Protein Response and Are Required for C. elegans Development

2001 • 693 citations

Translational control in the endoplasmic reticulum stress response

2002 • 686 citations

The unfolded protein response in nutrient sensing and differentiation

2002 • 592 citations

Coupling Endoplasmic Reticulum Stress to the Cell Death Program

2001 • 584 citations

The PERK Eukaryotic Initiation Factor 2α Kinase Is Required for the Development of the Skeletal System, Postnatal Growth, and the Function and Viability of the Pancreas

2002 • 584 citations

The Ca2+ concentration of the endoplasmic reticulum is a key determinant of ceramide-induced apoptosis: significance for the molecular mechanism of Bcl-2 action

2001 • 578 citations

Hyperhomocysteinemia enhances vascular inflammation and accelerates atherosclerosis in a murine model

2001 • 523 citations

Diverse functions of JNK signaling and c-Jun in stress response and apoptosis

1999 • 516 citations

Coupling Endoplasmic Reticulum Stress to the Cell Death Program

2002 • 480 citations

CHIP Is Associated with Parkin, a Gene Responsible for Familial Parkinson's Disease, and Enhances Its Ubiquitin Ligase Activity

2002 • 470 citations

Mammalian stress response: induction of the glucose-regulated protein family

1992 • 452 citations

Ligand-independent Dimerization Activates the Stress Response Kinases IRE1 and PERK in the Lumen of the Endoplasmic Reticulum

2000 • 408 citations

ER quality control: towards an understanding at the molecular level

2001 • 392 citations

Increased sensitivity to dextran sodium sulfate colitis in IRE1β-deficient mice

2001 • 390 citations

ATF6 as a Transcription Activator of the Endoplasmic Reticulum Stress Element: Thapsigargin Stress-Induced Changes and Synergistic Interactions with NF-Y and YY1

2000 • 345 citations

Molecular mechanisms and regulation of insulin exocytosis as a paradigm of endocrine secretion

1999 • 343 citations

Regulated Step in Cholesterol Feedback Localized to Budding of SCAP from ER Membranes

2000 • 332 citations

Translational control of proinsulin synthesis by glucose

1980 • 310 citations

Herp, a New Ubiquitin-like Membrane Protein Induced by Endoplasmic Reticulum Stress

2000 • 308 citations

A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.

1994 • 293 citations

Endoplasmic Reticulum Stress-Induced Formation of Transcription Factor Complex ERSF Including NF-Y (CBF) and Activating Transcription Factors 6α and 6β That Activates the Mammalian Unfolded Protein Response

2001 • 291 citations

Homocysteine Induces Programmed Cell Death in Human Vascular Endothelial Cells through Activation of the Unfolded Protein Response

2001 • 287 citations

A Role for Presenilin-1 in Nuclear Accumulation of Ire1 Fragments and Induction of the Mammalian Unfolded Protein Response

1999 • 281 citations

The endoribonuclease activity of mammalian IRE1 autoregulates its mRNA and is required for the unfolded protein response

2000 • 260 citations

Molecular Pathways of Protein Synthesis Inhibition during Brain Reperfusion: Implications for Neuronal Survival or Death

2002 • 236 citations

Overexpression of Pex15p, a phosphorylated peroxisomal integral membrane protein required for peroxisome assembly in S.cerevisiae, causes proliferation of the endoplasmic reticulum membrane

1997 • 221 citations

Brain ischemia and reperfusion activates the eukaryotic initiation factor 2α kinase, PERK

2001 • 214 citations

Dietary Supplementation With Methionine and Homocysteine Promotes Early Atherosclerosis but Not Plaque Rupture in ApoE-Deficient Mice

2001 • 213 citations

Procollagen folding and assembly: The role of endoplasmic reticulum enzymes and molecular chaperones

1999 • 203 citations

mRNA splicing-mediated C-terminal replacement of transcription factor Hac1p is required for efficient activation of the unfolded protein response

2000 • 181 citations

Protein aggregation in disease: a role for folding intermediates forming specific multimeric interactions

2002 • 175 citations

Preclinical and clinical evaluation of proteasome inhibitor PS-341 for the treatment of cancer

2002 • 144 citations

Homocysteine thiolactone induces apoptotic DNA damage mediated by increased intracellular hydrogen peroxide and caspase 3 activation in HL-60 cells

2001 • 130 citations

Targeting of the c-Abl Tyrosine Kinase to Mitochondria in Endoplasmic Reticulum Stress-Induced Apoptosis

2001 • 129 citations

Endoplasmic Reticulum Stress-inducible Protein, Herp, Enhances Presenilin-mediated Generation of Amyloid β-Protein

2002 • 124 citations

The Protein Kinase/Endoribonuclease IRE1α That Signals the Unfolded Protein Response Has a Luminal N-terminal Ligand-independent Dimerization Domain

2002 • 122 citations

The basic domain/leucine zipper protein hXBP-1 preferentially binds to and transactivates CRE-like sequences containing an ACGT core

1996 • 102 citations

Genetic tailoring of N-linked oligosaccharides: The role of glucose residues in glycoprotein processing of Saccharomyces cerevisiae in vivo

1998 • 87 citations

Targeted Disruption of CRE-Binding Factor TREB5 Gene Leads to Cellular Necrosis in Cardiac Myocytes at the Embryonic Stage

1999 • 65 citations

Cellular Origin of Hexokinase in Pancreatic Islets

1999 • 57 citations

Inducible Membranes in Yeast: Relation to the Unfolded-Protein-Response Pathway

1997 • 56 citations

Induction of glucose-regulated protein (glucose-regulated protein 78/B:P and glucose-regulated protein 94) and heat shock protein 70 transcripts in the immature rat brain following status epilepticus

1996 • 44 citations

Induction of secretory pathway components in yeast is associated with increased stability of their mRNA

2002 • 42 citations

Null Mutation in IRE1 Gene Inhibits Overproduction of Microsomal Cytochrome P450Alkl (CYP 52A3) and Proliferation of the Endoplasmic Reticulum in Saccharomyces cerevisiae

1999 • 26 citations

Ultrastructural changes during the enhancement of cellular 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase in a Chinese hamster cell mutant resistant to compactin (ML 236B).

1983 • 16 citations