Experts and Doctors on molecular chaperones in New York, United States


Locale: New York, United States
Topic: molecular chaperones

Top Publications

  1. Culi J, Mann R. Boca, an endoplasmic reticulum protein required for wingless signaling and trafficking of LDL receptor family members in Drosophila. Cell. 2003;112:343-54 pubmed
    ..Consequently, boca is an essential component of the Wingless pathway but is more generally required for the activities of multiple LDL receptor family members. ..
  2. Yang Z, Geng X, Solit D, Pratilas C, Rosen N, Danishefsky S. New efficient synthesis of resorcinylic macrolides via ynolides: establishment of cycloproparadicicol as synthetically feasible preclinical anticancer agent based on Hsp90 as the target. J Am Chem Soc. 2004;126:7881-9 pubmed
    ..The potency of their cytotoxicity was found to be consistent with their ability to degrade the oncogenic protein, Her2. From these assays, cycloproparadicicol was identified as a most promising candidate for further development. ..
  3. Heiman G, Ottman R, Saunders Pullman R, Ozelius L, Risch N, Bressman S. Increased risk for recurrent major depression in DYT1 dystonia mutation carriers. Neurology. 2004;63:631-7 pubmed
    ..These findings suggest that early-onset recurrent depression is a clinical expression of the DYT1 gene mutation. ..
  4. Massey A, Follenzi A, Kiffin R, Zhang C, Cuervo A. Early cellular changes after blockage of chaperone-mediated autophagy. Autophagy. 2008;4:442-56 pubmed
    ..Based on the time-course of the cellular changes, we propose that a minimal threshold of these toxic products needs to accumulate in order to constitutively activate macroautophagy and thus return cellular homeostasis to normal. ..
  5. Tanabe L, Kim C, Alagem N, Dauer W. Primary dystonia: molecules and mechanisms. Nat Rev Neurol. 2009;5:598-609 pubmed publisher
    ..Other dystonia-related gene products traffic through the endoplasmic reticulum, suggesting a potential cell biological theme underlying primary dystonia. ..
  6. Yi Z, Sperzel L, N rnberger C, Bredenbeek P, Lubick K, Best S, et al. Identification and characterization of the host protein DNAJC14 as a broadly active flavivirus replication modulator. PLoS Pathog. 2011;7:e1001255 pubmed publisher
    ..Our findings, together with previously published work, suggest that the members of the Flaviviridae family have evolved in unique and important ways to interact with this host Hsp40 chaperone molecule...
  7. Wilczek C, Chitta R, Woo E, Shabanowitz J, Chait B, Hunt D, et al. Protein arginine methyltransferase Prmt5-Mep50 methylates histones H2A and H4 and the histone chaperone nucleoplasmin in Xenopus laevis eggs. J Biol Chem. 2011;286:42221-31 pubmed publisher
  8. Silva A, Xu X, Kim H, Fillingham J, Kislinger T, Mennella T, et al. The replication-independent histone H3-H4 chaperones HIR, ASF1, and RTT106 co-operate to maintain promoter fidelity. J Biol Chem. 2012;287:1709-18 pubmed publisher
    ..We propose that nucleosome shielding is the primary means to maintain promoter fidelity, and histone replacement is most efficiently mediated in yeast cells by a HIR/Asf1/H3-H4/Rtt106 pathway. ..
  9. Xu J, Xu M, Brown T, Rossi G, Hurd Y, Inturrisi C, et al. Stabilization of the ?-opioid receptor by truncated single transmembrane splice variants through a chaperone-like action. J Biol Chem. 2013;288:21211-27 pubmed publisher
    ..Our studies suggest the functional roles of truncated receptors in other G protein-coupled receptor families. ..

More Information

Publications147 found, 100 shown here

  1. Melentijevic I, Tóth M, Arnold M, Guasp R, Harinath G, Nguyen K, et al. C. elegans neurons jettison protein aggregates and mitochondria under neurotoxic stress. Nature. 2017;542:367-371 pubmed publisher
  2. Anguiano J, Garner T, Mahalingam M, Das B, Gavathiotis E, Cuervo A. Chemical modulation of chaperone-mediated autophagy by retinoic acid derivatives. Nat Chem Biol. 2013;9:374-82 pubmed publisher
    ..We demonstrate that chemical enhancement of CMA protects cells from oxidative stress and from proteotoxicity, supporting a potential therapeutic opportunity when reduced CMA contributes to cellular dysfunction and disease. ..
  3. Chuang J, Zhou H, Zhu M, Li S, Li X, Sung C. Characterization of a brain-enriched chaperone, MRJ, that inhibits Huntingtin aggregation and toxicity independently. J Biol Chem. 2002;277:19831-8 pubmed
    ..Collectively, these results suggest that MRJ has a relevant functional role in neurons. ..
  4. Walker R, Good P, Shashidharan P. TorsinA immunoreactivity in inclusion bodies in trinucleotide repeat diseases. Mov Disord. 2003;18:1041-4 pubmed
  5. Einbond L, Su T, Wu H, Friedman R, Wang X, Jiang B, et al. Gene expression analysis of the mechanisms whereby black cohosh inhibits human breast cancer cell growth. Anticancer Res. 2007;27:697-712 pubmed
    ..Since the MeOH extract activated genes that enhance apoptosis and repressed cell cycle genes, it may be useful in the prevention and therapy of breast cancer. ..
  6. Vo A, Argyelan M, Eidelberg D, Ulug A. Early registration of diffusion tensor images for group tractography of dystonia patients. J Magn Reson Imaging. 2013;37:67-75 pubmed publisher
    ..This analysis visualized group level white matter fractional anisotropy and tract differences between dystonia patients and controls, and can be useful in understanding the pathophysiology of other nonfocal white matter diseases. ..
  7. Asuni A, Pankiewicz J, Sadowski M. Differential molecular chaperone response associated with various mouse adapted scrapie strains. Neurosci Lett. 2013;538:26-31 pubmed publisher
    ..Our results highlight the probability that different mechanisms of MCs regulation exist driven by amyloidogenic and non-amyloidogenic nature of prion strains. ..
  8. Okayama S, Kopelovich L, Balmus G, Weiss R, Herbert B, Dannenberg A, et al. p53 protein regulates Hsp90 ATPase activity and thereby Wnt signaling by modulating Aha1 expression. J Biol Chem. 2014;289:6513-25 pubmed publisher
    ..Taken together, this study provides new insights into the mechanism by which p53 regulates Wnt signaling and raises the intriguing possibility that p53 status may affect the efficacy of anticancer therapies targeting Hsp90 ATPase. ..
  9. Cheng H, Cenciarelli C, Shao Z, Vidal M, Parks W, Pagano M, et al. Human T cell leukemia virus type 1 Tax associates with a molecular chaperone complex containing hTid-1 and Hsp70. Curr Biol. 2001;11:1771-5 pubmed
    ..In the presence of Tax, hTid-1 and its associated Hsp70 are sequestered within a cytoplasmic "hot spot" structure, a subcellular distribution that is characteristic of Tax in HEK cells. ..
  10. Seigel G, Lotery A, Kummer A, Bernard D, Greene N, Turmaine M, et al. Retinal pathology and function in a Cln3 knockout mouse model of juvenile Neuronal Ceroid Lipofuscinosis (batten disease). Mol Cell Neurosci. 2002;19:515-27 pubmed
  11. Shen J, Chen X, Hendershot L, Prywes R. ER stress regulation of ATF6 localization by dissociation of BiP/GRP78 binding and unmasking of Golgi localization signals. Dev Cell. 2002;3:99-111 pubmed
    ..These results suggest that BiP retains ATF6 in the ER by inhibiting its GLSs and that dissociation of BiP during ER stress allows ATF6 to be transported to the Golgi. ..
  12. Pagant S, Kung L, DORRINGTON M, Lee M, Miller E. Inhibiting endoplasmic reticulum (ER)-associated degradation of misfolded Yor1p does not permit ER export despite the presence of a diacidic sorting signal. Mol Biol Cell. 2007;18:3398-413 pubmed
    ..We propose that the ER quality control checkpoint engages misfolded Yor1p even after it has been stabilized by inhibition of the degradative pathway...
  13. Johnson F, Kaplitt M. Novel mitochondrial substrates of omi indicate a new regulatory role in neurodegenerative disorders. PLoS ONE. 2009;4:e7100 pubmed publisher
    ..However higher dose treatment caused increased Omi expression and decreased levels of pdhb and idh3a transcripts. This implicates mitochondrial OMI in a novel mechanism relating to metabolism. ..
  14. Kim C, Perez A, Perkins G, Ellisman M, Dauer W. A molecular mechanism underlying the neural-specific defect in torsinA mutant mice. Proc Natl Acad Sci U S A. 2010;107:9861-6 pubmed publisher
  15. Rosenbaum A, Maxfield F. Niemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches. J Neurochem. 2011;116:789-95 pubmed publisher
    ..In this review, we discuss the possible mechanisms of action for NPC1 and NPC2 in mediating cholesterol efflux, as well as the different therapeutic approaches being pursued for the treatment of this lipid storage disorder. ..
  16. Gareau J, Reverter D, Lima C. Determinants of small ubiquitin-like modifier 1 (SUMO1) protein specificity, E3 ligase, and SUMO-RanGAP1 binding activities of nucleoporin RanBP2. J Biol Chem. 2012;287:4740-51 pubmed publisher
  17. Pearce D, Ferea T, Nosel S, Das B, Sherman F. Action of BTN1, the yeast orthologue of the gene mutated in Batten disease. Nat Genet. 1999;22:55-8 pubmed
    ..Our findings draw parallels between fundamental biological processes in yeast and previously observed characteristics of neurodegeneration in humans. ..
  18. Hon T, Lee H, Hach A, Johnson J, Craig E, Erdjument Bromage H, et al. The Hsp70-Ydj1 molecular chaperone represses the activity of the heme activator protein Hap1 in the absence of heme. Mol Cell Biol. 2001;21:7923-32 pubmed
    ..Our results suggest that Ssa-Ydj1 and Sro9 act together to mediate Hap1 repression in the absence of heme and that molecular chaperones promote heme regulation of Hap1 by a mechanism distinct from the mechanism of steroid signaling. ..
  19. Gupta P, Walter M, Su Z, Lebedeva I, Emdad L, Randolph A, et al. BiP/GRP78 is an intracellular target for MDA-7/IL-24 induction of cancer-specific apoptosis. Cancer Res. 2006;66:8182-91 pubmed
  20. Wisniewski T, Sadowski M. Preventing beta-amyloid fibrillization and deposition: beta-sheet breakers and pathological chaperone inhibitors. BMC Neurosci. 2008;9 Suppl 2:S5 pubmed publisher
    ..When combined with early pathology detection, these therapeutic strategies hold great promise as effective and relatively toxicity free methods of preventing AD related pathology. ..
  21. Santagata S, Bhattacharyya D, Wang F, Singha N, Hodtsev A, Spanopoulou E. Molecular cloning and characterization of a mouse homolog of bacterial ClpX, a novel mammalian class II member of the Hsp100/Clp chaperone family. J Biol Chem. 1999;274:16311-9 pubmed
    ..Our results thus suggest that murine ClpX acts as a tissue-specific mammalian mitochondrial chaperone that may play a role in mitochondrial protein homeostasis. ..
  22. Kang Y, Taldone T, Clement C, Fewell S, Aguirre J, Brodsky J, et al. Design of a fluorescence polarization assay platform for the study of human Hsp70. Bioorg Med Chem Lett. 2008;18:3749-51 pubmed publisher
    ..Here, we present a novel assay platform based on fluorescence polarization that is suitable for investigating the yet elusive molecular mechanics of human Hsp70 allosteric regulation. ..
  23. Shi Y, Chen Y, Dackour R, Potters L, Wang S, Ding Q, et al. Synuclein gamma stimulates membrane-initiated estrogen signaling by chaperoning estrogen receptor (ER)-alpha36, a variant of ER-alpha. Am J Pathol. 2010;177:964-73 pubmed publisher
  24. Stavropoulos P, Nagy V, Blobel G, Hoelz A. Molecular basis for the autoregulation of the protein acetyl transferase Rtt109. Proc Natl Acad Sci U S A. 2008;105:12236-41 pubmed publisher
    ..We propose a model in which the autoacetylation of Rtt109 is crucial for the regulation of its catalytic activity. ..
  25. Chahales P, Hoffman P, Thanassi D. Nitazoxanide Inhibits Pilus Biogenesis by Interfering with Folding of the Usher Protein in the Outer Membrane. Antimicrob Agents Chemother. 2016;60:2028-38 pubmed publisher
    ..This suggests that further development of the NTZ scaffold may lead to new antivirulence agents that target the usher to prevent pilus assembly. ..
  26. Matunis M, Wu J, Blobel G. SUMO-1 modification and its role in targeting the Ran GTPase-activating protein, RanGAP1, to the nuclear pore complex. J Cell Biol. 1998;140:499-509 pubmed
    ..This nuclear localization signal, and the presence of nine leucine-rich nuclear export signal motifs, suggests that RanGAP1 may shuttle between the nucleus and the cytoplasm. ..
  27. Hon T, Hach A, Tamalis D, Zhu Y, Zhang L. The yeast heme-responsive transcriptional activator Hap1 is a preexisting dimer in the absence of heme. J Biol Chem. 1999;274:22770-4 pubmed
    ..Hap1 repression is caused, at least in part, by inhibition of the DNA binding activity of the preexisting dimer. ..
  28. Jiang Y, Liu Y, Goldberg I, Shi Y. Gamma synuclein, a novel heat-shock protein-associated chaperone, stimulates ligand-dependent estrogen receptor alpha signaling and mammary tumorigenesis. Cancer Res. 2004;64:4539-46 pubmed
    ..These data indicate that SNCG is a new chaperone protein in the Hsp-based multiprotein chaperone complex for stimulation of ligand-dependent ER-alpha signaling and thus stimulates hormone-responsive mammary tumorigenesis. ..
  29. Lilic M, Vujanac M, Stebbins C. A common structural motif in the binding of virulence factors to bacterial secretion chaperones. Mol Cell. 2006;21:653-64 pubmed publisher
    ..Disruption of this structural motif leads to a destabilization of several chaperone-substrate complexes from different species, as well as an impairment of secretion in Salmonella...
  30. Yoneda T, Benedetti C, Urano F, Clark S, Harding H, Ron D. Compartment-specific perturbation of protein handling activates genes encoding mitochondrial chaperones. J Cell Sci. 2004;117:4055-66 pubmed
    ..These observations support the existence of a mitochondrial unfolded protein response that couples mitochondrial chaperone gene expression to changes in the protein handling environment in the organelle. ..
  31. Sharma N, Hewett J, Ozelius L, Ramesh V, McLean P, Breakefield X, et al. A close association of torsinA and alpha-synuclein in Lewy bodies: a fluorescence resonance energy transfer study. Am J Pathol. 2001;159:339-44 pubmed
    ..Using sensitive fluorescent resonance energy transfer (FRET) techniques, we find evidence of a close association between torsinA and alpha-synuclein in Lewy bodies. ..
  32. Décaillot F, Rozenfeld R, Gupta A, Devi L. Cell surface targeting of mu-delta opioid receptor heterodimers by RTP4. Proc Natl Acad Sci U S A. 2008;105:16045-50 pubmed publisher
    ..This has implications in the modulation of pharmacology of their endogenous ligands, and in the development of drugs with specific therapeutic effects. ..
  33. Doudevski I, Rostagno A, Cowman M, Liebmann J, Ritch R, Ghiso J. Clusterin and complement activation in exfoliation glaucoma. Invest Ophthalmol Vis Sci. 2014;55:2491-9 pubmed publisher
  34. Benedetti C, Haynes C, Yang Y, Harding H, Ron D. Ubiquitin-like protein 5 positively regulates chaperone gene expression in the mitochondrial unfolded protein response. Genetics. 2006;174:229-39 pubmed
    ..Induction of mitochondrial stress promotes accumulation of GFP-tagged UBL-5 in nuclei of transgenic worms, suggesting that UBL-5 effects a nuclear step required for mounting a response to the threat of mitochondrial protein misfolding. ..
  35. Solit D, Scher H, Rosen N. Hsp90 as a therapeutic target in prostate cancer. Semin Oncol. 2003;30:709-16 pubmed
    ..These data suggest that inhibitors of Hsp90 may represent a novel strategy for the treatment of patients with prostate cancer and clinical trials to test this hypothesis are currently ongoing. ..
  36. Kassube S, Stuwe T, Lin D, Antonuk C, Napetschnig J, Blobel G, et al. Crystal structure of the N-terminal domain of Nup358/RanBP2. J Mol Biol. 2012;423:752-65 pubmed publisher
    ..Together, these data suggest that the NTD contributes to mRNP remodeling events at the cytoplasmic face of the NPC. ..
  37. Goodchild R, Dauer W. The AAA+ protein torsinA interacts with a conserved domain present in LAP1 and a novel ER protein. J Cell Biol. 2005;168:855-62 pubmed
  38. Isaias I, Alterman R, Tagliati M. Deep brain stimulation for primary generalized dystonia: long-term outcomes. Arch Neurol. 2009;66:465-70 pubmed publisher
    ..Pallidal DBS is a safe and effective treatment for PGD, with improvement sustained for up to 8 years in 1 patient. Low energies of stimulation, although they did not affect clinical outcome, were associated with longer battery life. ..
  39. Tian G, Thomas S, Cowan N. Effect of TBCD and its regulatory interactor Arl2 on tubulin and microtubule integrity. Cytoskeleton (Hoboken). 2010;67:706-14 pubmed publisher
  40. Li S, Banck M, Mujtaba S, Zhou M, SUGRUE M, Walsh M. p53-induced growth arrest is regulated by the mitochondrial SirT3 deacetylase. PLoS ONE. 2010;5:e10486 pubmed publisher
    ..These studies identify a complex relationship between p53, SirT3, and chaperoning factor BAG-2 that may link the salvaging and quality assurance of the p53 protein for control of cellular fate independent of transcriptional activity. ..
  41. Carbon M, Argyelan M, Ghilardi M, Mattis P, Dhawan V, Bressman S, et al. Impaired sequence learning in dystonia mutation carriers: a genotypic effect. Brain. 2011;134:1416-27 pubmed publisher
    ..The close relationship between reduced cerebellar pathway integrity and increased learning-related activation of the premotor cortex is compatible with the view of DYT1 dystonia as a neurodevelopmental circuit disorder...
  42. Aung T, Ozaki M, Lee M, Schlötzer Schrehardt U, Thorleifsson G, Mizoguchi T, et al. Genetic association study of exfoliation syndrome identifies a protective rare variant at LOXL1 and five new susceptibility loci. Nat Genet. 2017;49:993-1004 pubmed publisher
    ..3 (TMEM136), 6p21 (AGPAT1), 3p24 (RBMS3) and 5q23 (near SEMA6A). These findings provide biological insights into the pathology of XFS and highlight a potential role for naturally occurring rare LOXL1 variants in disease biology. ..
  43. Hohfeld J, Minami Y, Hartl F. Hip, a novel cochaperone involved in the eukaryotic Hsc70/Hsp40 reaction cycle. Cell. 1995;83:589-98 pubmed
    ..We propose a mechanism for the regulation of eukaryotic Hsc70 that is distinct from that of bacterial Hsp70. The Hsc70/Hsp40/Hip system is apparently independent of a GrpE-like nucleotide exchange factor. ..
  44. Neiman A, Katz L, Brennwald P. Identification of domains required for developmentally regulated SNARE function in Saccharomyces cerevisiae. Genetics. 2000;155:1643-55 pubmed
    ..Deletion studies indicate that activation and inhibition are separable functions of the Spo20p N terminus. Our results reveal an additional layer of regulation of the SNARE complex, which is necessary only in sporulating cells. ..
  45. Mendes C, Levet C, Chatelain G, Dourlen P, Fouillet A, Dichtel Danjoy M, et al. ER stress protects from retinal degeneration. EMBO J. 2009;28:1296-307 pubmed publisher
    ..We propose that an immediate consequence of the UPR not only limits the accumulation of misfolded proteins but also protects tissues from harmful exogenous stresses. ..
  46. Gaitanaris G, Vysokanov A, Hung S, Gottesman M, Gragerov A. Successive action of Escherichia coli chaperones in vivo. Mol Microbiol. 1994;14:861-9 pubmed
    ..In contrast, GroEL, although it is transiently associated with newly synthesized proteins, is absent from the ribosomes. This suggests that DnaK and DnaJ play an early role in protein maturation, whereas GroEL acts at a later stage. ..
  47. Frydman J, Hartl F. Principles of chaperone-assisted protein folding: differences between in vitro and in vivo mechanisms. Science. 1996;272:1497-502 pubmed
    ..Thus, folding, oligomerization, and degradation are linked hierarchically to ensure the correct fate of newly synthesized polypeptides. ..
  48. Saitoh H, Pizzi M, Wang J. Perturbation of SUMOlation enzyme Ubc9 by distinct domain within nucleoporin RanBP2/Nup358. J Biol Chem. 2002;277:4755-63 pubmed
  49. Simons C, Staes A, Rommelaere H, Ampe C, Lewis S, Cowan N. Selective contribution of eukaryotic prefoldin subunits to actin and tubulin binding. J Biol Chem. 2004;279:4196-203 pubmed
    ..Our data are consistent with the hypothesis that PFD, like the eukaryotic cytosolic chaperonin, has co-evolved specifically to facilitate the folding of its target proteins. ..
  50. Ng T, Akman L, Osisami M, Thanassi D. The usher N terminus is the initial targeting site for chaperone-subunit complexes and participates in subsequent pilus biogenesis events. J Bacteriol. 2004;186:5321-31 pubmed
    ..Thus, the usher N terminus does not function simply as a static binding site for chaperone-subunit complexes but also participates in subsequent pilus assembly events. ..
  51. Ishii S, Yoshioka H, Mannen K, Kulkarni A, Fan J. Transgenic mouse expressing human mutant alpha-galactosidase A in an endogenous enzyme deficient background: a biochemical animal model for studying active-site specific chaperone therapy for Fabry disease. Biochim Biophys Acta. 2004;1690:250-7 pubmed
    ..These TgM/KO mice and TMK cells are useful tools for studying the mechanism of ASSC therapy, and for screening ASSCs for Fabry disease. ..
  52. Wang Y, Bogenhagen D. Human mitochondrial DNA nucleoids are linked to protein folding machinery and metabolic enzymes at the mitochondrial inner membrane. J Biol Chem. 2006;281:25791-802 pubmed
    ..Antibodies raised against a recombinant fragment of this protein confirmed the mitochondrial localization of a specific isoform of DHX30. ..
  53. Oyadomari S, Yun C, Fisher E, Kreglinger N, Kreibich G, Oyadomari M, et al. Cotranslocational degradation protects the stressed endoplasmic reticulum from protein overload. Cell. 2006;126:727-39 pubmed
    ..Thus, P58(IPK) is a key mediator of cotranslocational ER protein degradation, and this process likely contributes to ER homeostasis in stressed cells. ..
  54. Taibi A, Lighthouse J, Grady R, Shroyer K, Holdener B. Development of a conditional Mesd (mesoderm development) allele for functional analysis of the low-density lipoprotein receptor-related family in defined tissues. PLoS ONE. 2013;8:e75782 pubmed publisher
    ..This conditional allele will serve as an excellent tool for future characterization of the cumulative contribution of LRP members in defined tissues. ..
  55. Caplan A. Hsp90's secrets unfold: new insights from structural and functional studies. Trends Cell Biol. 1999;9:262-8 pubmed
    ..This review addresses recent developments in our understanding of the structure and function of Hsp90. ..
  56. Goodchild R, Dauer W. Mislocalization to the nuclear envelope: an effect of the dystonia-causing torsinA mutation. Proc Natl Acad Sci U S A. 2004;101:847-52 pubmed
    ..DYT1 dystonia appears to be a previously uncharacterized NE disease and the first, to our knowledge, to selectively affect CNS function...
  57. Oxelmark E, Roth J, Brooks P, Braunstein S, Schneider R, Garabedian M. The cochaperone p23 differentially regulates estrogen receptor target genes and promotes tumor cell adhesion and invasion. Mol Cell Biol. 2006;26:5205-13 pubmed
    ..Our findings demonstrate that p23 differentially regulates ER target genes and is involved in the control of distinct cellular processes in breast tumor development, thus revealing novel functions of this cochaperone. ..
  58. Armata I, Ananthanarayanan M, Balasubramaniyan N, Shashidharan P. Regulation of DYT1 gene expression by the Ets family of transcription factors. J Neurochem. 2008;106:1052-65 pubmed publisher
    ..Ets-like dominant negatives confirmed the Ets factors as DYT1 transcriptional activators. ..
  59. Cowan N, Lewis S. A chaperone with a hydrophilic surface. Nat Struct Biol. 1999;6:990-1 pubmed
    ..The structure of the yeast homolog of cofactor A, Rbl2p, shows it to be a dimer with largely hydrophilic surfaces, reflecting the fact that it interacts with quasi-native, not unfolded, beta-tubulin. ..
  60. Weimer J, Kriscenski Perry E, Elshatory Y, Pearce D. The neuronal ceroid lipofuscinoses: mutations in different proteins result in similar disease. Neuromolecular Med. 2002;1:111-24 pubmed
    ..We review the function of the CLN-proteins and discuss the possibility that a disruption in a common biological process leads to an NCL-disease. ..
  61. McNaught K, Kapustin A, Jackson T, Jengelley T, Jnobaptiste R, Shashidharan P, et al. Brainstem pathology in DYT1 primary torsion dystonia. Ann Neurol. 2004;56:540-7 pubmed
    ..and cuneiform nuclei, and related brainstem brainstem structures, in mediating motor activity and controlling muscle tone suggests that alterations in these structures could underlie the pathophysiology of DYT1 dystonia [corrected]..
  62. Stallings C, Silverstein S. Dissection of a novel nuclear localization signal in open reading frame 29 of varicella-zoster virus. J Virol. 2005;79:13070-81 pubmed
    ..As a result of this interaction, this noncanonical NLS appears to hijack the classical cellular nuclear import machinery. Elucidation of the mechanisms governing ORF29p nuclear targeting could shed light on the VZV reactivation process. ..
  63. Petrova K, Oyadomari S, Hendershot L, Ron D. Regulated association of misfolded endoplasmic reticulum lumenal proteins with P58/DNAJc3. EMBO J. 2008;27:2862-72 pubmed publisher
    ..These findings are consistent with a model whereby localized activation of the Hsp70-type partner is sufficient to promote substrate handover from the J-domain co-chaperone. ..
  64. Kuan L, Schaffer J, Zouzias C, Pearson M. Characterization of 17 chaperone-usher fimbriae encoded by Proteus mirabilis reveals strong conservation. J Med Microbiol. 2014;63:911-22 pubmed publisher
    ..These findings suggest there may be evolutionary pressure for P. mirabilis to maintain a large fimbrial arsenal...
  65. Harrison C, Hayer Hartl M, Di Liberto M, Hartl F, Kuriyan J. Crystal structure of the nucleotide exchange factor GrpE bound to the ATPase domain of the molecular chaperone DnaK. Science. 1997;276:431-5 pubmed
    ..This conformational change is not consistent with tight nucleotide binding. Two long alpha helices extend away from the GrpE dimer and suggest a role for GrpE in peptide release from DnaK. ..
  66. Basso A, Solit D, Chiosis G, Giri B, Tsichlis P, Rosen N. Akt forms an intracellular complex with heat shock protein 90 (Hsp90) and Cdc37 and is destabilized by inhibitors of Hsp90 function. J Biol Chem. 2002;277:39858-66 pubmed
    ..Thus, transduction of growth factor signaling via the Akt and Raf pathways requires functional Hsp90 and can be coordinately blocked by its inhibition. ..
  67. Haynes C, Ron D. The mitochondrial UPR - protecting organelle protein homeostasis. J Cell Sci. 2010;123:3849-55 pubmed publisher
    ..Here, we review the current knowledge of this mitochondrial unfolded protein response (UPR(mt)), compare it with the better understood UPR of the endoplasmic reticulum and highlight its potential impact on development and disease. ..
  68. Cheng C, Chen C, Feng Z, Marshall A, Bardin C. Rat clusterin isolated from primary Sertoli cell-enriched culture medium is sulfated glycoprotein-2 (SGP-2). Biochem Biophys Res Commun. 1988;155:398-404 pubmed
  69. Harding H, Novoa I, Zhang Y, Zeng H, Wek R, Schapira M, et al. Regulated translation initiation controls stress-induced gene expression in mammalian cells. Mol Cell. 2000;6:1099-108 pubmed
    ..Mammalian cells thus utilize an ancient pathway to regulate gene expression in response to diverse stress signals. ..
  70. Lee P, Rao J, Fliss A, Yang E, Garrett S, Caplan A. The Cdc37 protein kinase-binding domain is sufficient for protein kinase activity and cell viability. J Cell Biol. 2002;159:1051-9 pubmed
    ..This may be a form of regulation by which cells restrict access to Cdc37 until it has passed through a triage involving other chaperones such as Hsp70 and Hsp90. ..
  71. Ghilardi M, Carbon M, Silvestri G, Dhawan V, Tagliati M, Bressman S, et al. Impaired sequence learning in carriers of the DYT1 dystonia mutation. Ann Neurol. 2003;54:102-9 pubmed
    ..Although localized increases in neural activity may enable normal movement execution in these subjects, this mechanism may not compensate for their defect in sequence learning. ..
  72. Phelps Durr T, Thomas J, Vahab P, Timmermans M. Maize rough sheath2 and its Arabidopsis orthologue ASYMMETRIC LEAVES1 interact with HIRA, a predicted histone chaperone, to maintain knox gene silencing and determinacy during organogenesis. Plant Cell. 2005;17:2886-98 pubmed publisher
    ..Components of this process are conserved in animals, suggesting the possibility that a similar epigenetic mechanism maintains determinacy during both plant and animal development...
  73. Haynes C, Petrova K, Benedetti C, Yang Y, Ron D. ClpP mediates activation of a mitochondrial unfolded protein response in C. elegans. Dev Cell. 2007;13:467-80 pubmed
    ..As ClpP is known to function in the bacterial heat-shock response, our findings suggest that eukaryotes utilize component(s) from the protomitochondrial symbiont to signal the UPR(mt). ..
  74. Wang Y, Shen J, Arenzana N, Tirasophon W, Kaufman R, Prywes R. Activation of ATF6 and an ATF6 DNA binding site by the endoplasmic reticulum stress response. J Biol Chem. 2000;275:27013-20 pubmed
  75. Doherty K, Pride L, Lukose J, Snydsman B, Charles R, Pramanik A, et al. Loss of a 20S proteasome activator in Saccharomyces cerevisiae downregulates genes important for genomic integrity, increases DNA damage, and selectively sensitizes cells to agents with diverse mechanisms of action. G3 (Bethesda). 2012;2:943-59 pubmed publisher
    ..Targeting Blm10/PA200 together with one or more of the upregulated chaperones or a conventional treatment could be efficacious. ..
  76. Wong E, Cuervo A. Integration of clearance mechanisms: the proteasome and autophagy. Cold Spring Harb Perspect Biol. 2010;2:a006734 pubmed publisher
  77. Bali M, Zhang B, Morano K, Michels C. The Hsp90 molecular chaperone complex regulates maltose induction and stability of the Saccharomyces MAL gene transcription activator Mal63p. J Biol Chem. 2003;278:47441-8 pubmed
  78. Asanuma K, Carbon Correll M, Eidelberg D. Neuroimaging in human dystonia. J Med Invest. 2005;52 Suppl:272-9 pubmed
    ..This presentation will focus on an integrated approach to understanding the pathophysiology of this genetic and biochemically diverse disorder. ..
  79. Abul Husn N, Annangudi S, Ma ayan A, Ramos Ortolaza D, Stockton S, Gomes I, et al. Chronic morphine alters the presynaptic protein profile: identification of novel molecular targets using proteomics and network analysis. PLoS ONE. 2011;6:e25535 pubmed publisher
  80. Liu J, Hicks D, Krulwich T. Roles of AtpI and two YidC-type proteins from alkaliphilic Bacillus pseudofirmus OF4 in ATP synthase assembly and nonfermentative growth. J Bacteriol. 2013;195:220-30 pubmed publisher
    ..Deletion of atpZ caused the largest increase among the mutants in magnesium concentrations needed for pH 7.5 growth. The basis for this phenotype is not yet resolved. ..
  81. Kiryluk K, Li Y, Moldoveanu Z, Suzuki H, Reily C, Hou P, et al. GWAS for serum galactose-deficient IgA1 implicates critical genes of the O-glycosylation pathway. PLoS Genet. 2017;13:e1006609 pubmed publisher
  82. Vainberg I, Lewis S, Rommelaere H, Ampe C, Vandekerckhove J, Klein H, et al. Prefoldin, a chaperone that delivers unfolded proteins to cytosolic chaperonin. Cell. 1998;93:863-73 pubmed
    ..We show that by directing target proteins to chaperonin, prefoldin promotes folding in an environment in which there are many competing pathways for nonnative proteins. ..
  83. Knoblauch R, Garabedian M. Role for Hsp90-associated cochaperone p23 in estrogen receptor signal transduction. Mol Cell Biol. 1999;19:3748-59 pubmed
    ..Together, these results strongly suggest that p23 plays an important role in ER signal transduction. ..
  84. Beatrix B, Sakai H, Wiedmann M. The alpha and beta subunit of the nascent polypeptide-associated complex have distinct functions. J Biol Chem. 2000;275:37838-45 pubmed
    ..However, betaNAC alone directly binds to the ribosome and is sufficient to prevent ribosome binding to the endoplasmic reticulum membrane. ..
  85. Huang T, Deng H, Wolkoff A, Stockert R. Phosphorylation-dependent interaction of the asialoglycoprotein receptor with molecular chaperones. J Biol Chem. 2002;277:37798-803 pubmed
    ..The data presented provide evidence that phosphorylation of the ASGPR cytoplasmic domain is required for the binding of specific molecular chaperones with the potential to regulate receptor trafficking. ..
  86. Shah M, Patel K, Fried V, Sehgal P. Interactions of STAT3 with caveolin-1 and heat shock protein 90 in plasma membrane raft and cytosolic complexes. Preservation of cytokine signaling during fever. J Biol Chem. 2002;277:45662-9 pubmed
    ..Taken together, the data show that IL-6/raft/STAT3 signaling is a chaperoned pathway that involves cav-1 and HSP90 as accessory proteins and suggest a mechanism for the preservation of this signaling during fever. ..
  87. Doheny D, Danisi F, Smith C, Morrison C, Velickovic M, De Leon D, et al. Clinical findings of a myoclonus-dystonia family with two distinct mutations. Neurology. 2002;59:1244-6 pubmed
    ..Further analysis of these mutations separately and together in cell culture and in animal models should clarify their functional consequences. ..
  88. Carbon M, Trost M, Ghilardi M, Eidelberg D. Abnormal brain networks in primary torsion dystonia. Adv Neurol. 2004;94:155-61 pubmed
  89. Tzagoloff A, Barrientos A, Neupert W, Herrmann J. Atp10p assists assembly of Atp6p into the F0 unit of the yeast mitochondrial ATPase. J Biol Chem. 2004;279:19775-80 pubmed
    ..Based on these observations, we propose Atp10p to be an Atp6p-specific chaperone that facilitates the incorporation of Atp6p into an intermediate subcomplex of ATPase subunits. ..
  90. Chattopadhyay S, Kingsley E, Serour A, Curran T, Brooks A, Pearce D. Altered gene expression in the eye of a mouse model for batten disease. Invest Ophthalmol Vis Sci. 2004;45:2893-905 pubmed
    ..More detailed experimentation is needed to understand the contribution of these changes in expression to disease state, and whether these changes are specific for certain cell types within the eye. ..
  91. Dey A, Kessova I, Cederbaum A. Decreased protein and mRNA expression of ER stress proteins GRP78 and GRP94 in HepG2 cells over-expressing CYP2E1. Arch Biochem Biophys. 2006;447:155-66 pubmed
    ..These results suggest that CYP2E1 mediated oxidative stress downregulates the expression of GRP proteins in HepG2 cells and oxidative stress is an important mechanism in causing ER dysfunction in these cells. ..
  92. Collins M, Hendrickson W. Structural characterization of the Boca/Mesd maturation factors for LDL-receptor-type ? propeller domains. Structure. 2011;19:324-36 pubmed publisher