Published in Physiol Rev on October 01, 2007
One step at a time: endoplasmic reticulum-associated degradation. Nat Rev Mol Cell Biol (2008) 8.68
Comprehensive characterization of genes required for protein folding in the endoplasmic reticulum. Science (2009) 4.58
The recognition and retrotranslocation of misfolded proteins from the endoplasmic reticulum. Traffic (2008) 3.07
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Peripheral protein quality control removes unfolded CFTR from the plasma membrane. Science (2010) 2.92
The insulin paradox: aging, proteotoxicity and neurodegeneration. Nat Rev Neurosci (2008) 2.10
Somatic mutations in angiopoietin receptor gene TEK cause solitary and multiple sporadic venous malformations. Nat Genet (2008) 1.95
Receptor quality control in the endoplasmic reticulum for plant innate immunity. EMBO J (2009) 1.94
Orm1 and Orm2 are conserved endoplasmic reticulum membrane proteins regulating lipid homeostasis and protein quality control. Proc Natl Acad Sci U S A (2010) 1.92
Defining the glycan destruction signal for endoplasmic reticulum-associated degradation. Mol Cell (2008) 1.86
Stringent requirement for HRD1, SEL1L, and OS-9/XTP3-B for disposal of ERAD-LS substrates. J Cell Biol (2010) 1.85
Palmitoylation and ubiquitination regulate exit of the Wnt signaling protein LRP6 from the endoplasmic reticulum. Proc Natl Acad Sci U S A (2008) 1.64
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Malfolded protein structure and proteostasis in lung diseases. Am J Respir Crit Care Med (2014) 1.47
Ubiquilin and p97/VCP bind erasin, forming a complex involved in ERAD. J Cell Biol (2009) 1.45
Crystal structure of Miner1: The redox-active 2Fe-2S protein causative in Wolfram Syndrome 2. J Mol Biol (2009) 1.41
Substrate-specific mediators of ER associated degradation (ERAD). Curr Opin Cell Biol (2009) 1.40
A dual task for the Xbp1-responsive OS-9 variants in the mammalian endoplasmic reticulum: inhibiting secretion of misfolded protein conformers and enhancing their disposal. J Biol Chem (2008) 1.38
Substrate discrimination of the chaperone BiP by autonomous and cochaperone-regulated conformational transitions. Nat Struct Mol Biol (2011) 1.38
Uncoupling of sustained MAMP receptor signaling from early outputs in an Arabidopsis endoplasmic reticulum glucosidase II allele. Proc Natl Acad Sci U S A (2009) 1.36
Protein folding and quality control in the ER. Cold Spring Harb Perspect Biol (2011) 1.33
Protein folding in the cell: challenges and progress. Curr Opin Struct Biol (2010) 1.32
Identification and characterization of pharmacological chaperones to correct enzyme deficiencies in lysosomal storage disorders. Assay Drug Dev Technol (2011) 1.28
The neuroprotective mechanism of brain ischemic preconditioning. Acta Pharmacol Sin (2009) 1.27
Glaucoma-associated myocilin: a better understanding but much more to learn. Exp Eye Res (2008) 1.24
Molecular chaperones and substrate ubiquitination control the efficiency of endoplasmic reticulum-associated degradation. Diabetes Obes Metab (2010) 1.23
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Sarcoglycanopathies: molecular pathogenesis and therapeutic prospects. Expert Rev Mol Med (2009) 1.22
Protein quality control and degradation in cardiomyocytes. J Mol Cell Cardiol (2008) 1.22
Disulfide bonds in ER protein folding and homeostasis. Curr Opin Cell Biol (2010) 1.17
The endoplasmic reticulum-associated degradation of the epithelial sodium channel requires a unique complement of molecular chaperones. Mol Biol Cell (2010) 1.17
ERp57 modulates STAT3 signaling from the lumen of the endoplasmic reticulum. J Biol Chem (2009) 1.16
The Polyomaviridae: Contributions of virus structure to our understanding of virus receptors and infectious entry. Virology (2009) 1.15
Targeting Huntington's disease through histone deacetylases. Clin Epigenetics (2011) 1.13
Retrotranslocation of prion proteins from the endoplasmic reticulum by preventing GPI signal transamidation. Mol Biol Cell (2008) 1.12
Control of cellular GADD34 levels by the 26S proteasome. Mol Cell Biol (2008) 1.09
Subcellular stress response and induction of molecular chaperones and folding proteins after transient global ischemia in rats. Brain Res (2008) 1.08
Ero1-α and PDIs constitute a hierarchical electron transfer network of endoplasmic reticulum oxidoreductases. J Cell Biol (2013) 1.07
Darwinian selection for sites of Asn-linked glycosylation in phylogenetically disparate eukaryotes and viruses. Proc Natl Acad Sci U S A (2009) 1.06
Structural and functional relationships between the lectin and arm domains of calreticulin. J Biol Chem (2011) 1.05
A highly conserved motif at the COOH terminus dictates endoplasmic reticulum exit and cell surface expression of NKCC2. J Biol Chem (2009) 1.04
Pharmacological Chaperone Therapy: Preclinical Development, Clinical Translation, and Prospects for the Treatment of Lysosomal Storage Disorders. Mol Ther (2015) 1.04
Pharmacochaperone-mediated rescue of calcium-sensing receptor loss-of-function mutants. Mol Endocrinol (2009) 1.04
Trypanosoma brucei UDP-glucose:glycoprotein glucosyltransferase has unusual substrate specificity and protects the parasite from stress. Eukaryot Cell (2008) 1.04
The ubiquitin ligase E6-AP is induced and recruited to aggresomes in response to proteasome inhibition and may be involved in the ubiquitination of Hsp70-bound misfolded proteins. J Biol Chem (2009) 1.00
ERdj4 protein is a soluble endoplasmic reticulum (ER) DnaJ family protein that interacts with ER-associated degradation machinery. J Biol Chem (2012) 1.00
The VP1 subunit of JC polyomavirus recapitulates early events in viral trafficking and is a novel tool to study polyomavirus entry. Virology (2012) 1.00
Lectin chaperones help direct the maturation of glycoproteins in the endoplasmic reticulum. Biochim Biophys Acta (2009) 0.99
The mammalian UPR boosts glycoprotein ERAD by suppressing the proteolytic downregulation of ER mannosidase I. J Cell Sci (2009) 0.98
Orchestration of secretory protein folding by ER chaperones. Biochim Biophys Acta (2013) 0.98
Effects of cellular, chemical, and pharmacological chaperones on the rescue of a trafficking-defective mutant of the ATP-binding cassette transporter proteins ABCB1/ABCB4. J Biol Chem (2011) 0.97
Rescue of expression and signaling of human luteinizing hormone G protein-coupled receptor mutants with an allosterically binding small-molecule agonist. Proc Natl Acad Sci U S A (2011) 0.97
N-linked sugar-regulated protein folding and quality control in the ER. Semin Cell Dev Biol (2014) 0.97
Prion protein biosynthesis and its emerging role in neurodegeneration. Trends Biochem Sci (2009) 0.97
Calnexin is not essential for mammalian rod opsin biogenesis. Mol Vis (2008) 0.97
Importance of post-translational modifications for functionality of a chloroplast-localized carbonic anhydrase (CAH1) in Arabidopsis thaliana. PLoS One (2011) 0.97
Protein misfolding and retinal degeneration. Cold Spring Harb Perspect Biol (2011) 0.96
Proteasome malfunction activates macroautophagy in the heart. Am J Cardiovasc Dis (2011) 0.96
An interaction map of endoplasmic reticulum chaperones and foldases. Mol Cell Proteomics (2012) 0.96
Novel thioredoxin-related transmembrane protein TMX4 has reductase activity. J Biol Chem (2010) 0.95
ER stress: Autophagy induction, inhibition and selection. Autophagy (2015) 0.95
Hepatic lipase maturation: a partial proteome of interacting factors. J Lipid Res (2009) 0.95
Neuroligin trafficking deficiencies arising from mutations in the alpha/beta-hydrolase fold protein family. J Biol Chem (2010) 0.95
Calnexin deficiency leads to dysmyelination. J Biol Chem (2010) 0.94
Targeting histone deacetylases for the treatment of disease. J Cell Mol Med (2008) 0.94
Single molecule fluorescence approaches shed light on intracellular RNAs. Chem Rev (2014) 0.94
Endoplasmic reticulum stress in the absence of calnexin. Cell Stress Chaperones (2008) 0.93
Differential rates of protein folding and cellular trafficking for the Hendra virus F and G proteins: implications for F-G complex formation. J Virol (2009) 0.93
The endoplasmic reticulum chaperone Cosmc directly promotes in vitro folding of T-synthase. J Biol Chem (2009) 0.93
N-Glycan-based ER Molecular Chaperone and Protein Quality Control System: The Calnexin Binding Cycle. Traffic (2016) 0.93
The cotranslational maturation program for the type II membrane glycoprotein influenza neuraminidase. J Biol Chem (2008) 0.93
The intrinsic and extrinsic effects of N-linked glycans on glycoproteostasis. Nat Chem Biol (2014) 0.93
Pro370Leu mutant myocilin impairs mitochondrial functions in human trabecular meshwork cells. Mol Vis (2009) 0.93
Interplay between the ubiquitin-proteasome system and autophagy in proteinopathies. Int J Physiol Pathophysiol Pharmacol (2009) 0.92
Autophagy and the ubiquitin-proteasome system in cardiac dysfunction. Panminerva Med (2010) 0.92
Cyclosporine A-sensitive, cyclophilin B-dependent endoplasmic reticulum-associated degradation. PLoS One (2010) 0.92
ERAD and how viruses exploit it. Front Microbiol (2014) 0.91
Endoplasmic reticulum stress-mediated activation of p38 MAPK, Caspase-2 and Caspase-8 leads to abrin-induced apoptosis. PLoS One (2014) 0.91
Endoplasmic reticulum stress, genome damage, and cancer. Front Oncol (2015) 0.90
Consequences of individual N-glycan deletions and of proteasomal inhibition on secretion of active BACE. Mol Biol Cell (2008) 0.90
Activation of ERAD pathway by human hepatitis B virus modulates viral and subviral particle production. PLoS One (2012) 0.89
The peripheral neuropathy-linked Trembler and Trembler-J mutant forms of peripheral myelin protein 22 are folding-destabilized. Biochemistry (2008) 0.89
Differences in endoplasmic-reticulum quality control determine the cellular response to disease-associated mutants of proteolipid protein. J Cell Sci (2009) 0.89
A cell-based reglucosylation assay demonstrates the role of GT1 in the quality control of a maturing glycoprotein. J Cell Biol (2008) 0.89
Calreticulin binds to gentamicin and reduces drug-induced ototoxicity. Toxicol Sci (2011) 0.88
Characterization of early EDEM1 protein maturation events and their functional implications. J Biol Chem (2011) 0.88
Mechanisms of lipase maturation. Clin Lipidol (2010) 0.88
The role of UDP-Glc:glycoprotein glucosyltransferase 1 in the maturation of an obligate substrate prosaposin. J Cell Biol (2010) 0.88
Transcript and in silico analysis of CLN3 in juvenile neuronal ceroid lipofuscinosis and associated mouse models. Hum Mol Genet (2008) 0.88
A cytosolic relay of heat shock proteins HSP70-1A and HSP90β monitors the folding trajectory of the serotonin transporter. J Biol Chem (2014) 0.88
Translational toxicology and rescue strategies of the hERG channel dysfunction: biochemical and molecular mechanistic aspects. Acta Pharmacol Sin (2014) 0.88
mTOR inhibition increases cell viability via autophagy induction during endoplasmic reticulum stress - An experimental and modeling study. FEBS Open Bio (2014) 0.87
Cotranslational and posttranslocational N-glycosylation of proteins in the endoplasmic reticulum. Semin Cell Dev Biol (2014) 0.87
A novel role for Gtb1p in glucose trimming of N-linked glycans. Glycobiology (2009) 0.87
Mammal-restricted elements predispose human RET to folding impairment by HSCR mutations. Nat Struct Mol Biol (2010) 0.87
Endoplasmic reticulum stress in amelogenesis imperfecta and phenotypic rescue using 4-phenylbutyrate. Hum Mol Genet (2013) 0.87
INS-gene mutations: from genetics and beta cell biology to clinical disease. Mol Aspects Med (2014) 0.86
Endoplasmic reticulum-associated degradation of a degron-containing polytopic membrane protein. Mol Membr Biol (2009) 0.86
Coronaviruses Hijack the LC3-I-positive EDEMosomes, ER-derived vesicles exporting short-lived ERAD regulators, for replication. Cell Host Microbe (2010) 2.75
N-glycan structures: recognition and processing in the ER. Trends Biochem Sci (2009) 2.26
The glycan code of the endoplasmic reticulum: asparagine-linked carbohydrates as protein maturation and quality-control tags. Trends Cell Biol (2005) 1.98
Stringent requirement for HRD1, SEL1L, and OS-9/XTP3-B for disposal of ERAD-LS substrates. J Cell Biol (2010) 1.85
N-glycan processing in ER quality control. J Cell Sci (2006) 1.64
A novel stress-induced EDEM variant regulating endoplasmic reticulum-associated glycoprotein degradation. J Biol Chem (2004) 1.64
EDEM1 regulates ER-associated degradation by accelerating de-mannosylation of folding-defective polypeptides and by inhibiting their covalent aggregation. Biochem Biophys Res Commun (2006) 1.46
ERAD substrates: which way out? Semin Cell Dev Biol (2009) 1.44
A dual task for the Xbp1-responsive OS-9 variants in the mammalian endoplasmic reticulum: inhibiting secretion of misfolded protein conformers and enhancing their disposal. J Biol Chem (2008) 1.38
Degradation of trafficking-defective long QT syndrome type II mutant channels by the ubiquitin-proteasome pathway. J Biol Chem (2005) 1.35
N-linked glycan recognition and processing: the molecular basis of endoplasmic reticulum quality control. Curr Opin Struct Biol (2006) 1.30
Segregation and rapid turnover of EDEM1 by an autophagy-like mechanism modulates standard ERAD and folding activities. Biochem Biophys Res Commun (2008) 1.28
Consequences of ERp57 deletion on oxidative folding of obligate and facultative clients of the calnexin cycle. J Biol Chem (2006) 1.27
Glycoprotein folding and the role of EDEM1, EDEM2 and EDEM3 in degradation of folding-defective glycoproteins. FEBS Lett (2007) 1.23
Early postnatal death and motor disorders in mice congenitally deficient in calnexin expression. Mol Cell Biol (2002) 1.22
ERAD and ERAD tuning: disposal of cargo and of ERAD regulators from the mammalian ER. Curr Opin Cell Biol (2011) 1.15
beta-site specific intrabodies to decrease and prevent generation of Alzheimer's Abeta peptide. J Cell Biol (2005) 1.14
Substrate-specific requirements for UGT1-dependent release from calnexin. Mol Cell (2007) 1.12
Flagging and docking: dual roles for N-glycans in protein quality control and cellular proteostasis. Trends Biochem Sci (2012) 1.09
Retracted Role of the SEL1L:LC3-I complex as an ERAD tuning receptor in the mammalian ER. Mol Cell (2012) 1.06
Malectin participates in a backup glycoprotein quality control pathway in the mammalian ER. PLoS One (2011) 1.04
Specificity and regulation of the endoplasmic reticulum-associated degradation machinery. Traffic (2013) 1.01
EDEM contributes to maintenance of protein folding efficiency and secretory capacity. J Biol Chem (2004) 0.97
The use of calnexin and calreticulin by cellular and viral glycoproteins. J Biol Chem (2005) 0.93
Autophagy-independent LC3 function in vesicular traffic. Autophagy (2010) 0.93
Cyclosporine A-sensitive, cyclophilin B-dependent endoplasmic reticulum-associated degradation. PLoS One (2010) 0.92
Consequences of individual N-glycan deletions and of proteasomal inhibition on secretion of active BACE. Mol Biol Cell (2008) 0.90
Microbiology: death of a chaperone. Nature (2006) 0.88
Unconventional use of LC3 by coronaviruses through the alleged subversion of the ERAD tuning pathway. Viruses (2011) 0.85
The disulphide bonds in the catalytic domain of BACE are critical but not essential for amyloid precursor protein processing activity. J Neurochem (2002) 0.83
UDP-glucose:glycoprotein glucosyltransferase (UGGT1) promotes substrate solubility in the endoplasmic reticulum. Mol Biol Cell (2013) 0.83
The endoplasmic reticulum crossroads for newly synthesized polypeptide chains. Prog Mol Biol Transl Sci (2008) 0.80
Transgenic expression of β1 antibody in brain neurons impairs age-dependent amyloid deposition in APP23 mice. Neurobiol Aging (2013) 0.79
Unconventional roles of nonlipidated LC3 in ERAD tuning and coronavirus infection. Autophagy (2012) 0.79
Analyzing folding and degradation of metabolically labelled polypeptides by conventional and diagonal sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Biol Proced Online (2005) 0.76
Chronic delivery of antibody fragments using immunoisolated cell implants as a passive vaccination tool. PLoS One (2011) 0.75
Corrigendum: Translocon component Sec62 acts in endoplasmic reticulum turnover during stress recovery. Nat Cell Biol (2016) 0.75