Molecular Chaperones

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  Subject Areas on Research

  • A Chlamydia trachomatis strain with a chemically generated amino acid substitution (P370L) in the cthtrA gene shows reduced elementary body production. 
  • A Role for Dystonia-Associated Genes in Spinal GABAergic Interneuron Circuitry. 
  • A galactosylceramide binding domain is involved in trafficking of CLN3 from Golgi to rafts via recycling endosomes. 
  • A novel receptor function for the heat shock protein Grp78: silencing of Grp78 gene expression attenuates alpha2M*-induced signalling. 
  • A novel, non-apoptotic role for Scythe/BAT3: a functional switch between the pro- and anti-proliferative roles of p21 during the cell cycle. 
  • Activation and cross-talk between Akt, NF-kappaB, and unfolded protein response signaling in 1-LN prostate cancer cells consequent to ligation of cell surface-associated GRP78. 
  • Altered telomeres in tumors with ATRX and DAXX mutations. 
  • An Unusual Presentation of CLN3-Associated Batten Disease With Classic Histopathologic and Ultrastructural Findings. 
  • Anti-tumor necrosis factor-alpha therapy augments dipeptidyl peptidase IV activity and decreases autoantibodies to GRP78/BIP and phosphoglucose isomerase in patients with rheumatoid arthritis. 
  • Apatorsen plus docetaxel versus docetaxel alone in platinum-resistant metastatic urothelial carcinoma (Borealis-2). 
  • Apolipoprotein J/clusterin induction in myocarditis: A localized response gene to myocardial injury. 
  • Association of the kinesin-binding domain of RanBP2 to KIF5B and KIF5C determines mitochondria localization and function. 
  • Bat3 facilitates H3K79 dimethylation by DOT1L and promotes DNA damage-induced 53BP1 foci at G1/G2 cell-cycle phases. 
  • Binding of activated alpha2-macroglobulin to its cell surface receptor GRP78 in 1-LN prostate cancer cells regulates PAK-2-dependent activation of LIMK. 
  • Biochemical, cell biological and immunological issues surrounding the endoplasmic reticulum chaperone GRP94/gp96. 
  • Bortezomib inhibits PKR-like endoplasmic reticulum (ER) kinase and induces apoptosis via ER stress in human pancreatic cancer cells. 
  • CHOP mediates endoplasmic reticulum stress-induced apoptosis in Gimap5-deficient T cells. 
  • CLN3p impacts galactosylceramide transport, raft morphology, and lipid content. 
  • CNS1 encodes an essential p60/Sti1 homolog in Saccharomyces cerevisiae that suppresses cyclophilin 40 mutations and interacts with Hsp90. 
  • Calreticulin chaperones regulate functional expression of vomeronasal type 2 pheromone receptors. 
  • Calreticulin displays in vivo peptide-binding activity and can elicit CTL responses against bound peptides. 
  • Changes in macroautophagy, chaperone-mediated autophagy, and mitochondrial metabolism in murine skeletal and cardiac muscle during aging. 
  • Chaperone proteins and brain tumors: potential targets and possible therapeutics. 
  • Chaperone-mediated cross-priming: a hitchhiker's guide to vesicle transport (review). 
  • Chaperones and disease. 
  • Chaperones rescue luciferase folding by separating its domains. 
  • Characterization of RanBP2-associated molecular components in neuroretina. 
  • Clusterin promotes the aggregation and adhesion of renal porcine epithelial cells. 
  • Clusterin: an enigmatic protein recruited by diverse stimuli. 
  • Comparative analysis of the ATP-binding sites of Hsp90 by nucleotide affinity cleavage: a distinct nucleotide specificity of the C-terminal ATP-binding site. 
  • Cyclophilin-related protein RanBP2 acts as chaperone for red/green opsin. 
  • DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. 
  • DYT-TOR1A subcellular proteomics reveals selective vulnerability of the nuclear proteome to cell stress. 
  • Differential DNA methylation patterns in human Schlemm's canal endothelial cells with glaucoma. 
  • Differential loss of prolyl isomerase or chaperone activity of Ran-binding protein 2 (Ranbp2) unveils distinct physiological roles of its cyclophilin domain in proteostasis. 
  • Differential production of high molecular weight sulfated glycoproteins in normal colonic mucosa, primary colon carcinoma, and metastases. 
  • Distinct and atypical intrinsic and extrinsic cell death pathways between photoreceptor cell types upon specific ablation of Ranbp2 in cone photoreceptors. 
  • Endoplasmic reticulum chaperone GRP94 subunit assembly is regulated through a defined oligomerization domain. 
  • Endoplasmic reticulum stress. 
  • Evidence that alpha-crystallin prevents non-specific protein aggregation in the intact eye lens. 
  • Expression of clusterin in human renal diseases. 
  • Flupirtine blocks apoptosis in batten patient lymphoblasts and in human postmitotic CLN3- and CLN2-deficient neurons. 
  • From Drosophila to humans: reflections on the roles of the prolyl isomerases and chaperones, cyclophilins, in cell function and disease. 
  • Functional Genomic Analyses of Mendelian and Sporadic Disease Identify Impaired eIF2α Signaling as a Generalizable Mechanism for Dystonia. 
  • Functional complexity and regulation through RNA dynamics. 
  • Functional evidence implicating a novel TOR1A mutation in idiopathic, late-onset focal dystonia. 
  • Functional promoter rs2868371 variant of HSPB1 associates with radiation-induced esophageal toxicity in patients with non-small-cell lung cancer treated with radio(chemo)therapy. 
  • Functional promoter variant rs2868371 of HSPB1 is associated with risk of radiation pneumonitis after chemoradiation for non-small cell lung cancer. 
  • Functional roles of the two domains of phosducin and phosducin-like protein. 
  • GRP78: a chaperone with diverse roles beyond the endoplasmic reticulum. 
  • Genetic association study of exfoliation syndrome identifies a protective rare variant at LOXL1 and five new susceptibility loci. 
  • Genetic, biochemical, and structural studies of biogenesis of adhesive pili in bacteria. 
  • Genomic organization, expression, and localization of murine Ran-binding protein 2 (RanBP2) gene. 
  • Gp93, the Drosophila GRP94 ortholog, is required for gut epithelial homeostasis and nutrient assimilation-coupled growth control. 
  • HDAC6 controls major cell response pathways to cytotoxic accumulation of protein aggregates. 
  • HLA-B-associated transcript 3 (Bat3)/Scythe is essential for p300-mediated acetylation of p53. 
  • Haploinsufficiency of RanBP2 is neuroprotective against light-elicited and age-dependent degeneration of photoreceptor neurons. 
  • Heat shock protein 47 : a chaperone for the fibrous cap? 
  • Heterotrimeric Galphaq11 co-immunoprecipitates with surface-anchored GRP78 from plasma membranes of alpha2M*-stimulated macrophages. 
  • Hsp70 and the Cochaperone StiA (Hop) Orchestrate Hsp90-Mediated Caspofungin Tolerance in Aspergillus fumigatus. 
  • HspR is a global negative regulator of heat shock gene expression in Deinococcus radiodurans. 
  • Hypothermic treatment restores glucose regulated protein 78 (GRP78) expression in ischemic brain. 
  • ISO: a critical evaluation of the role of peptides in heat shock/chaperone protein-mediated tumor rejection. 
  • Identification of RanBP2- and kinesin-mediated transport pathways with restricted neuronal and subcellular localization. 
  • Identification of a novel gene linked to parkin via a bi-directional promoter. 
  • Identification of novel quaternary domain interactions in the Hsp90 chaperone, GRP94. 
  • Immortalized rat proximal tubule cells produce membrane bound and soluble megalin. 
  • Impairments in age-dependent ubiquitin proteostasis and structural integrity of selective neurons by uncoupling Ran GTPase from the Ran-binding domain 3 of Ranbp2 and identification of novel mitochondrial isoforms of ubiquitin-conjugating enzyme E2I (ubc9) and Ranbp2. 
  • Induction of clusterin in acute and chronic oxidative renal disease in the rat and its dissociation from cell injury. 
  • Induction of p38MAPK and HSP27 phosphorylation in pemphigus patient skin. 
  • Induction of the unfolded protein response drives enhanced metabolism and chemoresistance in glioma cells. 
  • Inhibition of translation and induction of apoptosis by Bunyaviral nonstructural proteins bearing sequence similarity to reaper. 
  • Interaction of the polyglutamine protein ataxin-3 with Rad23 regulates toxicity in Drosophila models of Spinocerebellar Ataxia Type 3. 
  • Interconversion of red opsin isoforms by the cyclophilin-related chaperone protein Ran-binding protein 2. 
  • Interleukin-1beta mediates human airway epithelial cell migration via NF-kappaB. 
  • InvB is a type III secretion chaperone specific for SspA. 
  • Involvement of an ATP-dependent peptide chaperone in cross-presentation after DNA immunization. 
  • Kinesin-1 and mitochondrial motility control by discrimination of structurally equivalent but distinct subdomains in Ran-GTP-binding domains of Ran-binding protein 2. 
  • Ligation of cell surface-associated glucose-regulated protein 78 by receptor-recognized forms of alpha 2-macroglobulin: activation of p21-activated protein kinase-2-dependent signaling in murine peritoneal macrophages. 
  • Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes. 
  • M1 of Murine Gamma-Herpesvirus 68 Induces Endoplasmic Reticulum Chaperone Production. 
  • Manassantin A inhibits tumour growth under hypoxia through the activation of chaperone-mediated autophagy by modulating Hsp90 activity. 
  • Mechanistic Insight into the Suppression of Polyglutamine Aggregation by SRCP1. 
  • Microglial activation in an amyotrophic lateral sclerosis-like model caused by Ranbp2 loss and nucleocytoplasmic transport impairment in retinal ganglion neurons. 
  • Mining copper transport genes. 
  • Mitochondrial microproteins link metabolic cues to respiratory chain biogenesis. 
  • Modulation of the unfolded protein response by GRP78 in prostate cancer. 
  • Motifs within the CLN3 protein: modulation of cell growth rates and apoptosis. 
  • Mouse model of rare TOR1A variant found in sporadic focal dystonia impairs domains affected in DYT1 dystonia patients and animal models. 
  • Mutation of TBCE causes hypoparathyroidism-retardation-dysmorphism and autosomal recessive Kenny-Caffey syndrome. 
  • Mutations affecting the cytoplasmic functions of the co-chaperone DNAJB6 cause limb-girdle muscular dystrophy. 
  • Mutations in SCO2 are associated with autosomal-dominant high-grade myopia. 
  • NCP activates chloroplast transcription by controlling phytochrome-dependent dual nuclear and plastidial switches. 
  • NMR structure note: solution structure of the core domain of MESD that is essential for proper folding of LRP5/6. 
  • Neuroprotection resulting from insufficiency of RANBP2 is associated with the modulation of protein and lipid homeostasis of functionally diverse but linked pathways in response to oxidative stress. 
  • New therapeutic approaches for Pompe disease: enzyme replacement therapy and beyond. 
  • Overexpression of the glucose-regulated stress gene GRP78 in malignant but not benign human breast lesions. 
  • Parkin is not regulated by the unfolded protein response in human neuroblastoma cells. 
  • Peripheral noxious stimulation induces phosphorylation of the NMDA receptor NR1 subunit at the PKC-dependent site, serine-896, in spinal cord dorsal horn neurons. 
  • Phospholipid membrane composition affects EGF receptor and Notch signaling through effects on endocytosis during Drosophila development. 
  • Piecewise All-Atom SMD Simulations Reveal Key Secondary Structures in Luciferase Unfolding Pathway. 
  • Plasminogen structural domains exhibit different functions when associated with cell surface GRP78 or the voltage-dependent anion channel. 
  • Polymer hydrogels: Chaperoning vaccines. 
  • Prevention of brefeldin A-induced resistance to teniposide by the proteasome inhibitor MG-132: involvement of NF-kappaB activation in drug resistance. 
  • Prostate cancer cell proliferation in vitro is modulated by antibodies against glucose-regulated protein 78 isolated from patient serum. 
  • Protective responses in the ischemic myocardium. 
  • Protein fragment domains identified using 2D gel electrophoresis/MALDI-TOF. 
  • Protein kinase C signaling during T cell activation induces the endoplasmic reticulum stress response. 
  • Proteomic analysis of calcium/calmodulin-dependent protein kinase I and IV in vitro substrates reveals distinct catalytic preferences. 
  • Pseudomonas syringae pv. tomato DC3000 HopPtoM (CEL ORF3) is important for lesion formation but not growth in tomato and is secreted and translocated by the Hrp type III secretion system in a chaperone-dependent manner. 
  • Purification and characterization of astrocyte-secreted apolipoprotein E and J-containing lipoproteins from wild-type and human apoE transgenic mice. 
  • Purification and partial molecular characterization of GRP94, an ER resident chaperone. 
  • RANBP2 is an allosteric activator of the conventional kinesin-1 motor protein, KIF5B, in a minimal cell-free system. 
  • RPGRIP1s with distinct neuronal localization and biochemical properties associate selectively with RanBP2 in amacrine neurons. 
  • RanBP2 modulates Cox11 and hexokinase I activities and haploinsufficiency of RanBP2 causes deficits in glucose metabolism. 
  • Ranbp2 haploinsufficiency mediates distinct cellular and biochemical phenotypes in brain and retinal dopaminergic and glia cells elicited by the Parkinsonian neurotoxin, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). 
  • Reaper-induced dissociation of a Scythe-sequestered cytochrome c-releasing activity. 
  • Receptor mediated and fluid phase pathways for internalization of the ER Hsp90 chaperone GRP94 in murine macrophages. 
  • Regulation of the copper chaperone CCS by XIAP-mediated ubiquitination. 
  • Retina-specifically expressed novel subtypes of bovine cyclophilin. 
  • Reversible inhibition of Hsp70 chaperone function by Scythe and Reaper. 
  • Rewiring of Signaling Networks Modulating Thermotolerance in the Human Pathogen Cryptococcus neoformans. 
  • Ribosome exchange revisited: a mechanism for translation-coupled ribosome detachment from the ER membrane. 
  • Role of chaperones in antigen processing. 
  • SRCP1 Conveys Resistance to Polyglutamine Aggregation. 
  • SREC-I, a type F scavenger receptor, is an endocytic receptor for calreticulin. 
  • Safety and pharmacodynamic effects of a pharmacological chaperone on α-galactosidase A activity and globotriaosylceramide clearance in Fabry disease: report from two phase 2 clinical studies. 
  • Scythe: a novel reaper-binding apoptotic regulator. 
  • Sec63 and Xbp1 regulate IRE1α activity and polycystic disease severity. 
  • Selective impairment of a subset of Ran-GTP-binding domains of ran-binding protein 2 (Ranbp2) suffices to recapitulate the degeneration of the retinal pigment epithelium (RPE) triggered by Ranbp2 ablation. 
  • Sensitivity of mature Erbb2 to geldanamycin is conferred by its kinase domain and is mediated by the chaperone protein Hsp90. 
  • Solution structure and function of YndB, an AHSA1 protein from Bacillus subtilis. 
  • Solution structure of DFF40 and DFF45 N-terminal domain complex and mutual chaperone activity of DFF40 and DFF45. 
  • Specific regulation of noncanonical p38alpha activation by Hsp90-Cdc37 chaperone complex in cardiomyocyte. 
  • Stevens-Johnson syndrome induced by the cross-reactivity between teicoplanin and vancomycin. 
  • Structural basis of pilus subunit recognition by the PapD chaperone. 
  • Structures of AAA protein translocase Bcs1 suggest translocation mechanism of a folded protein. 
  • Synthetic effect between envelope stress and lack of outer membrane vesicle production in Escherichia coli. 
  • Targeting the cyclophilin domain of Ran-binding protein 2 (Ranbp2) with novel small molecules to control the proteostasis of STAT3, hnRNPA2B1 and M-opsin. 
  • The CLN3 gene is a novel molecular target for cancer drug discovery. 
  • The Chlamydia trachomatis type III secretion chaperone Slc1 engages multiple early effectors, including TepP, a tyrosine-phosphorylated protein required for the recruitment of CrkI-II to nascent inclusions and innate immune signaling. 
  • The Control Centers of Biomolecular Phase Separation: How Membrane Surfaces, PTMs, and Active Processes Regulate Condensation. 
  • The FNIP co-chaperones decelerate the Hsp90 chaperone cycle and enhance drug binding. 
  • The HIV protease inhibitor, ritonavir, corrects diverse brain phenotypes across development in mouse model of DYT-TOR1A dystonia. 
  • The Mechanism of HdeA Unfolding and Chaperone Activation. 
  • The N-terminal region of RTP1S plays important roles in dimer formation and odorant receptor-trafficking. 
  • The cyclophilin-like domain mediates the association of Ran-binding protein 2 with subunits of the 19 S regulatory complex of the proteasome. 
  • The cyclophilin-like domain of Ran-binding protein-2 modulates selectively the activity of the ubiquitin-proteasome system and protein biogenesis. 
  • The docking of kinesins, KIF5B and KIF5C, to Ran-binding protein 2 (RanBP2) is mediated via a novel RanBP2 domain. 
  • The glucocorticoid receptor interacting protein 1 (GRIP1) localizes in discrete nuclear foci that associate with ND10 bodies and are enriched in components of the 26S proteasome. 
  • The heat shock response and chaperones/heat shock proteins in brain tumors: surface expression, release, and possible immune consequences. 
  • The immunological properties of endoplasmic reticulum chaperones: a conflict of interest? 
  • The mitochondrial targeting chaperone 14-3-3ε regulates a RIG-I translocon that mediates membrane association and innate antiviral immunity. 
  • The role of Grp 78 in alpha 2-macroglobulin-induced signal transduction. Evidence from RNA interference that the low density lipoprotein receptor-related protein is associated with, but not necessary for, GRP 78-mediated signal transduction. 
  • The role of MTJ-1 in cell surface translocation of GRP78, a receptor for alpha 2-macroglobulin-dependent signaling. 
  • The scaffold protein WRAP53β orchestrates the ubiquitin response critical for DNA double-strand break repair. 
  • The yeast HSM3 gene is not involved in DNA mismatch repair in rapidly dividing cells. 
  • The zinc finger cluster domain of RanBP2 is a specific docking site for the nuclear export factor, exportin-1. 
  • Therapeutic aspects of chaperones/heat-shock proteins in neuro-oncology. 
  • Thermodynamic analysis of a molecular chaperone binding to unfolded protein substrates. 
  • To find the road traveled to tumor immunity: the trafficking itineraries of molecular chaperones in antigen-presenting cells. 
  • Transcription errors induce proteotoxic stress and shorten cellular lifespan. 
  • Transcription factor TFII-I causes transcriptional upregulation of GRP78 synthesis in prostate cancer cells. 
  • Transcriptional inhibition by carcinogenic chromate: relationship to DNA damage. 
  • Transethnic genome-wide scan identifies novel Alzheimer's disease loci. 
  • Transient regulation of c-fos, alpha B-crystallin, and hsp70 in muscle during recovery from contractile activity. 
  • Type III protein secretion in Pseudomonas syringae. 
  • Uncoupling phototoxicity-elicited neural dysmorphology and death by insidious function and selective impairment of Ran-binding protein 2 (Ranbp2). 
  • Unraveling the mysteries of chaperone interactions of the myosin head. 
  • Up-regulation of GRP78 and antiapoptotic signaling in murine peritoneal macrophages exposed to insulin. 
  • c-Abl Mediated Tyrosine Phosphorylation of Aha1 Activates Its Co-chaperone Function in Cancer Cells. 
  • p38-MAPK induced dephosphorylation of alpha-tropomyosin is associated with depression of myocardial sarcomeric tension and ATPase activity. 
  • p53-dependent induction of heat shock protein 27 (HSP27) expression. 
  • α-Hemoglobin stabilizing protein (AHSP) markedly decreases the redox potential and reactivity of α-subunits of human HbA with hydrogen peroxide. 
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  Keywords of People

  • Calakos, Nicole, Lincoln Financial Group Distinguished Professor of Neurobiology, Duke Science & Society
  • Ferreira, Paulo Alexandre, Associate Professor in Ophthalmology, Pathology
  • Kuehn, Margarethe Joanna, Associate Professor of Biochemistry, Cell Biology