Published in Chem Biol on August 14, 2014
Unfolded protein response-induced ERdj3 secretion links ER stress to extracellular proteostasis. EMBO J (2014) 0.96
Close and Allosteric Opening of the Polypeptide-Binding Site in a Human Hsp70 Chaperone BiP. Structure (2015) 0.88
Regulating extracellular proteostasis capacity through the unfolded protein response. Prion (2015) 0.80
Converging roles of ion channels, calcium, metabolic stress, and activity pattern of Substantia nigra dopaminergic neurons in health and Parkinson's disease. J Neurochem (2016) 0.77
Mutant glucocerebrosidase in Gaucher disease recruits Hsp27 to the Hsp90 chaperone complex for proteasomal degradation. Proc Natl Acad Sci U S A (2015) 0.76
The endoplasmic reticulum HSP40 co-chaperone ERdj3/DNAJB11 assembles and functions as a tetramer. EMBO J (2017) 0.75
Rehabilitating mutant GCase. Chem Biol (2014) 0.75
Lysosomal Enzyme Glucocerebrosidase Protects against Aβ1-42 Oligomer-Induced Neurotoxicity. PLoS One (2015) 0.75
How Do J-Proteins Get Hsp70 to Do So Many Different Things? Trends Biochem Sci (2017) 0.75
Mapping and Exploring the Collagen-I Proteostasis Network. ACS Chem Biol (2016) 0.75
Erdj3 has an essential role for Z variant Alpha-1-Antitrypsin Degradation. J Cell Biochem (2017) 0.75
Stabilization of glucocerebrosidase by active-site occupancy. ACS Chem Biol (2017) 0.75
Stable isotope labeling by amino acids in cell culture, SILAC, as a simple and accurate approach to expression proteomics. Mol Cell Proteomics (2002) 30.58
Large-scale analysis of the yeast proteome by multidimensional protein identification technology. Nat Biotechnol (2001) 28.01
Global quantification of mammalian gene expression control. Nature (2011) 22.36
Adapting proteostasis for disease intervention. Science (2008) 13.22
XBP-1 regulates a subset of endoplasmic reticulum resident chaperone genes in the unfolded protein response. Mol Cell Biol (2003) 11.17
One step at a time: endoplasmic reticulum-associated degradation. Nat Rev Mol Cell Biol (2008) 8.68
Transcriptional induction of mammalian ER quality control proteins is mediated by single or combined action of ATF6alpha and XBP1. Dev Cell (2007) 5.45
The HSP70 chaperone machinery: J proteins as drivers of functional specificity. Nat Rev Mol Cell Biol (2010) 5.37
Biological and chemical approaches to diseases of proteostasis deficiency. Annu Rev Biochem (2009) 5.23
The cell biology of lysosomal storage disorders. Nat Rev Mol Cell Biol (2004) 3.89
Gaucher disease: mutation and polymorphism spectrum in the glucocerebrosidase gene (GBA). Hum Mutat (2008) 3.53
LIMP-2 is a receptor for lysosomal mannose-6-phosphate-independent targeting of beta-glucocerebrosidase. Cell (2007) 3.49
Chemical and biological approaches synergize to ameliorate protein-folding diseases. Cell (2008) 3.11
ATF6 is a transcription factor specializing in the regulation of quality control proteins in the endoplasmic reticulum. Cell Struct Funct (2008) 2.42
Chemical chaperones and permissive temperatures alter localization of Gaucher disease associated glucocerebrosidase variants. ACS Chem Biol (2006) 2.25
Genetic heterogeneity in type 1 Gaucher disease: multiple genotypes in Ashkenazic and non-Ashkenazic individuals. Proc Natl Acad Sci U S A (1988) 2.10
Molecular determinants of drug binding and action on L-type calcium channels. Annu Rev Pharmacol Toxicol (1997) 2.06
ER retention and degradation as the molecular basis underlying Gaucher disease heterogeneity. Hum Mol Genet (2005) 2.01
Common and uncommon pathogenic cascades in lysosomal storage diseases. J Biol Chem (2010) 1.85
Viable mouse models of acid beta-glucosidase deficiency: the defect in Gaucher disease. Am J Pathol (2003) 1.81
Glycoprotein folding, quality control and ER-associated degradation. Curr Opin Struct Biol (2009) 1.74
Protein folding and quality control in the endoplasmic reticulum: Recent lessons from yeast and mammalian cell systems. Curr Opin Cell Biol (2011) 1.74
Protein quality control in the ER: the recognition of misfolded proteins. Semin Cell Dev Biol (2010) 1.69
ERdj3, a stress-inducible endoplasmic reticulum DnaJ homologue, serves as a cofactor for BiP's interactions with unfolded substrates. Mol Biol Cell (2004) 1.66
Analysis of human acid beta-glucosidase by site-directed mutagenesis and heterologous expression. J Biol Chem (1994) 1.52
Impaired trafficking of mutants of lysosomal glucocerebrosidase in Gaucher's disease. Int J Biochem Cell Biol (2005) 1.50
HEDJ, an Hsp40 co-chaperone localized to the endoplasmic reticulum of human cells. J Biol Chem (2000) 1.50
ERAD substrates: which way out? Semin Cell Dev Biol (2009) 1.44
Partial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasis. PLoS Biol (2008) 1.42
Gaucher disease: gene frequencies and genotype/phenotype correlations. Genet Test (1997) 1.35
Elevation of intracellular glucosylceramide levels results in an increase in endoplasmic reticulum density and in functional calcium stores in cultured neurons. J Biol Chem (1999) 1.29
Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease. J Inherit Metab Dis (2004) 1.26
Endoplasmic reticulum Ca2+ increases enhance mutant glucocerebrosidase proteostasis. Nat Chem Biol (2010) 1.26
Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits. Hum Mol Genet (2010) 1.25
Enhanced calcium release in the acute neuronopathic form of Gaucher disease. Neurobiol Dis (2005) 1.20
Glucosylceramide and glucosylsphingosine modulate calcium mobilization from brain microsomes via different mechanisms. J Biol Chem (2003) 1.18
The endoplasmic reticulum-associated degradation of the epithelial sodium channel requires a unique complement of molecular chaperones. Mol Biol Cell (2010) 1.17
Gaucher disease: Perspectives on a prototype lysosomal disease. Cell Mol Life Sci (2002) 1.09
Characterization of high-affinity ryanodine-binding sites of rat liver endoplasmic reticulum. Differences between liver and skeletal muscle. Biochem J (1991) 1.08
ERdj3, a luminal ER DnaJ homologue, binds directly to unfolded proteins in the mammalian ER: identification of critical residues. Biochemistry (2009) 1.08
Monoclonal antibodies against human beta-glucocerebrosidase. Eur J Biochem (1983) 1.08
A procedure for the rapid purification in high yield of human glucocerebrosidase using immunoaffinity chromatography with monoclonal antibodies. Anal Biochem (1986) 1.06
Gaucher disease mutations in non-Jewish patients. Br J Haematol (1993) 1.06
Animal models for Gaucher disease research. Dis Model Mech (2011) 1.02
Chemical and/or biological therapeutic strategies to ameliorate protein misfolding diseases. Curr Opin Cell Biol (2010) 0.98
New strategies for the treatment of lysosomal storage diseases (review). Int J Mol Med (2012) 0.96
FKBP10 depletion enhances glucocerebrosidase proteostasis in Gaucher disease fibroblasts. Chem Biol (2013) 0.92
Ca2+ homeostasis modulation enhances the amenability of L444P glucosylcerebrosidase to proteostasis regulation in patient-derived fibroblasts. ACS Chem Biol (2010) 0.90
Lacidipine remodels protein folding and Ca 2+ homeostasis in Gaucher's disease fibroblasts: a mechanism to rescue mutant glucocerebrosidase. Chem Biol (2011) 0.89
Gaucher disease paradigm: from ERAD to comorbidity. Hum Mutat (2012) 0.89
Calcium channel blockers as antihypertensive agents. Am J Med (1984) 0.81
In vivo and ex vivo evaluation of L-type calcium channel blockers on acid beta-glucosidase in Gaucher disease mouse models. PLoS One (2009) 0.81
Comparative studies of calcium-channel blockers and beta-blockers in essential hypertension: clinical implications. Circulation (1987) 0.77
Detection of mutant protein in complex biological samples: glucocerebrosidase mutations in Gaucher's disease. Anal Biochem (2007) 0.77
Microglia promote learning-dependent synapse formation through brain-derived neurotrophic factor. Cell (2013) 3.91
Product binding enforces the genomic specificity of a yeast polycomb repressive complex. Cell (2014) 0.97
Isolation of chromatin from dysfunctional telomeres reveals an important role for Ring1b in NHEJ-mediated chromosome fusions. Cell Rep (2014) 0.95
Monoclonal 1- and 3-Phosphohistidine Antibodies: New Tools to Study Histidine Phosphorylation. Cell (2015) 0.88
Co-opting the Fanconi anemia genomic stability pathway enables herpesvirus DNA synthesis and productive growth. Mol Cell (2014) 0.83
Census 2: isobaric labeling data analysis. Bioinformatics (2014) 0.80
The Deubiquitylase MATH-33 Controls DAF-16 Stability and Function in Metabolism and Longevity. Cell Metab (2015) 0.78
Circadian Amplitude Regulation via FBXW7-Targeted REV-ERBα Degradation. Cell (2016) 0.78
A ribonuclease coordinates siRNA amplification and mRNA cleavage during RNAi. Cell (2015) 0.78
The Pallbearer E3 ligase promotes actin remodeling via RAC in efferocytosis by degrading the ribosomal protein S6. Dev Cell (2014) 0.78
Intracellular Action of a Secreted Peptide Required for Fungal Virulence. Cell Host Microbe (2016) 0.77
Noncanoncial signal recognition particle RNAs in a major eukaryotic phylum revealed by purification of SRP from the human pathogen Cryptococcus neoformans. Nucleic Acids Res (2015) 0.75
Glycolytic Enzymes Coalesce in G Bodies under Hypoxic Stress. Cell Rep (2017) 0.75