Protein Folding in the Endoplasmic Reticulum and the Unfolded Protein Response

Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism

1998 • 5,063 citations

Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease

1991 • 4,533 citations

Protein folding in the cell

1992 • 4,138 citations

Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease

1995 • 3,985 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

Endoplasmic Reticulum Stress Links Obesity, Insulin Action, and Type 2 Diabetes

2004 • 3,671 citations

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

2000 • 3,241 citations

Protein translation and folding are coupled by an endoplasmic-reticulum-resident kinase

1999 • 3,119 citations

An Integrated Stress Response Regulates Amino Acid Metabolism and Resistance to Oxidative Stress

2003 • 3,079 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

Dynamic interaction of BiP and ER stress transducers in the unfolded-protein response

2000 • 2,696 citations

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

1999 • 2,237 citations

Familial Alzheimer's disease in kindreds with missense mutations in a gene on chromosome 1 related to the Alzheimer's disease type 3 gene

1995 • 2,028 citations

XBP-1 Regulates a Subset of Endoplasmic Reticulum Resident Chaperone Genes in the Unfolded Protein Response

2003 • 1,979 citations

CHOP is implicated in programmed cell death in response to impaired function of the endoplasmic reticulum

1998 • 1,972 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

Perk Is Essential for Translational Regulation and Cell Survival during the Unfolded Protein Response

2000 • 1,933 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

Oligomerization and phosphorylation of the Ire1p kinase during intracellular signaling from the endoplasmic reticulum to the nucleus.

1996 • 1,306 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

Transcriptional induction of genes encoding endoplasmic reticulum resident proteins requires a transmembrane protein kinase

1993 • 1,197 citations

Coupling endoplasmic reticulum stress to the cell death program

2004 • 1,172 citations

Orchestrating the unfolded protein response in health and disease

2002 • 1,163 citations

Cross-Talk between Two Cysteine Protease Families

2000 • 1,158 citations

The AAA ATPase Cdc48/p97 and its partners transport proteins from the ER into the cytosol

2001 • 1,088 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

XBP1, Downstream of Blimp-1, Expands the Secretory Apparatus and Other Organelles, and Increases Protein Synthesis in Plasma Cell Differentiation

2004 • 978 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

Stress-Induced Phosphorylation and Activation of the Transcription Factor CHOP (GADD153) by p38 MAP Kinase

1996 • 893 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

The Transmembrane Kinase Ire1p Is a Site-Specific Endonuclease That Initiates mRNA Splicing in the Unfolded Protein Response

1997 • 886 citations

An Endoplasmic Reticulum Stress-specific Caspase Cascade in Apoptosis

2002 • 875 citations

Immunoglobulin heavy chain binding protein

1983 • 849 citations

Plasma cell differentiation and the unfolded protein response intersect at the transcription factor XBP-1

2003 • 843 citations

Activation of Caspase-12, an Endoplastic Reticulum (ER) Resident Caspase, through Tumor Necrosis Factor Receptor-associated Factor 2-dependent Mechanism in Response to the ER Stress

2001 • 832 citations

A transmembrane protein with a cdc2+CDC28-related kinase activity is required for signaling from the ER to the nucleus

1993 • 814 citations

A Familial Alzheimer's Disease Locus on Chromosome 1

1995 • 810 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

Identification and Characterization of Pancreatic Eukaryotic Initiation Factor 2 α-Subunit Kinase, PEK, Involved in Translational Control

1998 • 763 citations

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

2000 • 757 citations

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

2001 • 693 citations

Signals from the Stressed Endoplasmic Reticulum Induce C/EBP-Homologous Protein (CHOP/GADD153)

1996 • 671 citations

A Time-Dependent Phase Shift in the Mammalian Unfolded Protein Response

2003 • 670 citations

Two Distinct Stress Signaling Pathways Converge Upon the CHOP Promoter During the Mammalian Unfolded Protein Response

2002 • 666 citations

Retro-translocation of proteins from the endoplasmic reticulum into the cytosol

2002 • 637 citations

XBP1

2004 • 627 citations

How N-linked oligosaccharides affect glycoprotein folding in the endoplasmic reticulum.

1994 • 617 citations

Coupling Endoplasmic Reticulum Stress to the Cell Death Program

2001 • 584 citations

Protein dislocation from the ER requires polyubiquitination and the AAA-ATPase Cdc48

2002 • 530 citations

Presenilin-1 mutations downregulate the signalling pathway of the unfolded-protein response

1999 • 521 citations

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

1999 • 516 citations

Distinct roles of activating transcription factor 6 (ATF6) and double-stranded RNA-activated protein kinase-like endoplasmic reticulum kinase (PERK) in transcription during the mammalian unfolded protein response

2002 • 508 citations

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

2002 • 470 citations

Role of EDEM in the Release of Misfolded Glycoproteins from the Calnexin Cycle

2003 • 464 citations

Hrd1p/Der3p is a membrane-anchored ubiquitin ligase required for ER-associated degradation

2000 • 462 citations

A novel ER α‐mannosidase‐like protein accelerates ER‐associated degradation

2001 • 459 citations

A regulatory link between ER-associated protein degradation and the unfolded-protein response.

2000 • 459 citations

Proteasome-dependent endoplasmic reticulum-associated protein degradation: An unconventional route to a familiar fate

1996 • 451 citations

EDEM As an Acceptor of Terminally Misfolded Glycoproteins Released from Calnexin

2003 • 446 citations

Coordinated regulation of a set of genes by glucose and calcium ionophores in mammalian cells

1987 • 442 citations

tRNA Ligase Is Required for Regulated mRNA Splicing in the Unfolded Protein Response

1996 • 440 citations

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

2000 • 408 citations

A 22 bp cis-acting element is necessary and sufficient for the induction of the yeast KAR2 (BiP) gene by unfolded proteins.

1992 • 375 citations

The relationship of N-linked glycosylation and heavy chain-binding protein association with the secretion of glycoproteins.

1987 • 357 citations

Sequential Waves of Functionally Related Proteins Are Expressed When B Cells Prepare for Antibody Secretion

2003 • 351 citations

Overexpression of GRP78 mitigates stress induction of glucose regulated proteins and blocks secretion of selective proteins in Chinese hamster ovary cells.

1992 • 348 citations

Immunoglobulin Binding Protein (BiP) Function Is Required to Protect Cells from Endoplasmic Reticulum Stress but Is Not Required for the Secretion of Selective Proteins

1997 • 346 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

Translational control of proinsulin synthesis by glucose

1980 • 310 citations

The unfolded protein response sensor IRE1α is required at 2 distinct steps in B cell lymphopoiesis

2005 • 302 citations

Endoplasmic Reticulum Degradation Requires Lumen to Cytosol Signaling

2000 • 302 citations

Activation of an Unfolded Protein Response during Differentiation of Antibody-secreting B Cells

2002 • 297 citations

Role of ATP and disulphide bonds during protein folding in the endoplasmic reticulum

1992 • 286 citations

Induction of neuronal death by ER stress in Alzheimer’s disease

2004 • 284 citations

The unfolded protein response sensor IRE1α is required at 2 distinct steps in B cell lymphopoiesis

2005 • 280 citations

Induction of two transformation-sensitive membrane polypeptides in normal fibroblasts by a block in glycoprotein synthesis or glucose deprivation

1977 • 273 citations

5 Mechanism and Regulation of Initiator Methionyl-tRNA Binding to Ribosomes

2000 • 246 citations

Membrane biogenesis during B cell differentiation: most endoplasmic reticulum proteins are expressed coordinately.

1990 • 226 citations

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

2001 • 214 citations

Increased Vulnerability of Hippocampal Neurons from Presenilin‐1 Mutant Knock‐In Mice to Amyloid β‐Peptide Tox

1999 • 195 citations

Disturbed Activation of Endoplasmic Reticulum Stress Transducers by Familial Alzheimer's Disease-linked Presenilin-1 Mutations

2001 • 180 citations

The Unfolded Protein Response Pathway in

1996 • 180 citations

Regulation of mRNA translation by protein folding in the endoplasmic reticulum

2004 • 162 citations

Reduction of Endogenous GRP78 Levels Improves Secretion of a Heterologous Protein in CHO Cells

1988 • 152 citations

Biochemical characterization of the 94- and 78-kilodalton glucose-regulated proteins in hamster fibroblasts.

1984 • 148 citations

Protein dissociation from GRP78 and secretion are blocked by depletion of cellular ATP levels.

1990 • 147 citations

Recognition of local glycoprotein misfolding by the ER folding sensor UDP-glucose:glycoprotein glucosyltransferase.

2000 • 141 citations

Orchestrating the unfolded protein response in health and disease

2002 • 131 citations

Transcriptional regulation of two genes specifically induced by glucose starvation in a hamster mutant fibroblast cell line.

1983 • 128 citations

Increased Production of β-Amyloid and Vulnerability to Endoplasmic Reticulum Stress by an Aberrant Spliced Form of Presenilin 2

2001 • 119 citations

In Vivo Action of the HRD Ubiquitin Ligase Complex: Mechanisms of Endoplasmic Reticulum Quality Control and Sterol Regulation

2001 • 119 citations

Structure and Intermolecular Interactions of the Luminal Dimerization Domain of Human IRE1α

2003 • 115 citations

Transient Cerebral Ischemia Activates Processing of xbp1 Messenger RNA Indicative of Endoplasmic Reticulum Stress

2003 • 107 citations

Reduction of endogenous GRP78 levels improves secretion of a heterologous protein in CHO cells.

1988 • 104 citations

Unconventional Splicing of HAC1/ERN4 mRNA Required for the Unfolded Protein Response

1998 • 92 citations

Effect of Glucose on Initiation and Elongation Rates in Isolated Rat Pancreatic Islets

1974 • 58 citations

A role for the unfolded protein response in optimizing antibody secretion

2004 • 45 citations