Claudio Soto

Summary

Affiliation: Serono Pharmaceutical Research Institute
Country: Switzerland

Publications

  1. ncbi request reprint Diagnosing prion diseases: needs, challenges and hopes
    Claudio Soto
    Department of Neurology, University of Texas Medical Branch, 301 University Boulevard, Galveston, Texas 77555 0646, USA
    Nat Rev Microbiol 2:809-19. 2004
  2. ncbi request reprint Plaque busters: strategies to inhibit amyloid formation in Alzheimer's disease
    C Soto
    University of Chile Dept of Biology, Faculty of Sciences, Las Palmeras 3425, Santiago, Chile
    Mol Med Today 5:343-50. 1999
  3. ncbi request reprint Unfolding the role of protein misfolding in neurodegenerative diseases
    Claudio Soto
    Serono International, 15 Chemin des Mines, Geneva, Switzerland
    Nat Rev Neurosci 4:49-60. 2003
  4. ncbi request reprint Altering prion replication for therapy and diagnosis of transmissible spongiform encephalopathies
    C Soto
    Serono International, Geneva, Switzerland
    Biochem Soc Trans 30:569-74. 2002
  5. ncbi request reprint Cyclic amplification of protein misfolding: application to prion-related disorders and beyond
    Claudio Soto
    Serono International SA, Geneva, Switzerland
    Trends Neurosci 25:390-4. 2002
  6. ncbi request reprint Protein misfolding and disease; protein refolding and therapy
    C Soto
    Serono Pharmaceutical Research Institute, 14 Chemin des Aulx, 1228 Plan les Ouates, Geneva, Switzerland
    FEBS Lett 498:204-7. 2001
  7. ncbi request reprint Prions: disease propagation and disease therapy by conformational transmission
    C Soto
    Serono Pharmaceutical Research Institute, 14 Chemin des Aulx, 1228 Plan les Ouates, Geneva, Switzerland
    Trends Mol Med 7:109-14. 2001
  8. ncbi request reprint Inhibiting the conversion of soluble amyloid-beta peptide into abnormally folded amyloidogenic intermediates: relevance for Alzheimer's disease therapy
    C Soto
    Serono Pharmaceutical Research Institute, Geneva, Switzerland
    Acta Neurol Scand Suppl 176:90-5. 2000
  9. ncbi request reprint The disulfide isomerase Grp58 is a protective factor against prion neurotoxicity
    Claudio Hetz
    Serono Pharmaceutical Research Institute, 1228 Plan les Ouates, Geneva, Switzerland
    J Neurosci 25:2793-802. 2005
  10. ncbi request reprint Pharmacological profiles of peptide drug candidates for the treatment of Alzheimer's disease
    Celine Adessi
    Serono Pharmaceutical Research Institute, 1228 Geneva, Switzerland
    J Biol Chem 278:13905-11. 2003

Collaborators

Detail Information

Publications44

  1. ncbi request reprint Diagnosing prion diseases: needs, challenges and hopes
    Claudio Soto
    Department of Neurology, University of Texas Medical Branch, 301 University Boulevard, Galveston, Texas 77555 0646, USA
    Nat Rev Microbiol 2:809-19. 2004
    ..In this article, state-of-the-art prion diagnostic techniques, together with the new strategies that are being used to develop sensitive, early and non-invasive diagnoses for these diseases are reviewed...
  2. ncbi request reprint Plaque busters: strategies to inhibit amyloid formation in Alzheimer's disease
    C Soto
    University of Chile Dept of Biology, Faculty of Sciences, Las Palmeras 3425, Santiago, Chile
    Mol Med Today 5:343-50. 1999
    ..The effect of these and other compounds now needs to be tested in vivo and the ability of amyloid inhibitors to halt the progression of Alzheimer's disease in humans needs to be evaluated...
  3. ncbi request reprint Unfolding the role of protein misfolding in neurodegenerative diseases
    Claudio Soto
    Serono International, 15 Chemin des Mines, Geneva, Switzerland
    Nat Rev Neurosci 4:49-60. 2003
  4. ncbi request reprint Altering prion replication for therapy and diagnosis of transmissible spongiform encephalopathies
    C Soto
    Serono International, Geneva, Switzerland
    Biochem Soc Trans 30:569-74. 2002
    ..In this article, the potential use of the principles of prion replication to develop novel therapeutic and diagnostic strategies for these devastating diseases are described...
  5. ncbi request reprint Cyclic amplification of protein misfolding: application to prion-related disorders and beyond
    Claudio Soto
    Serono International SA, Geneva, Switzerland
    Trends Neurosci 25:390-4. 2002
    ..In this article we describe the rational behind PMCA and some of the many potential applications of this novel technology...
  6. ncbi request reprint Protein misfolding and disease; protein refolding and therapy
    C Soto
    Serono Pharmaceutical Research Institute, 14 Chemin des Aulx, 1228 Plan les Ouates, Geneva, Switzerland
    FEBS Lett 498:204-7. 2001
    ..Many questions are still pending, but the data overall suggest that correction of protein misfolding constitutes a viable therapeutic strategy for conformational diseases...
  7. ncbi request reprint Prions: disease propagation and disease therapy by conformational transmission
    C Soto
    Serono Pharmaceutical Research Institute, 14 Chemin des Aulx, 1228 Plan les Ouates, Geneva, Switzerland
    Trends Mol Med 7:109-14. 2001
    ..Here, we describe exciting scientific findings that make the prion field attractively heretical, and we propose the transmission of protein conformation as a novel approach to producing drugs to combat a variety of diseases...
  8. ncbi request reprint Inhibiting the conversion of soluble amyloid-beta peptide into abnormally folded amyloidogenic intermediates: relevance for Alzheimer's disease therapy
    C Soto
    Serono Pharmaceutical Research Institute, Geneva, Switzerland
    Acta Neurol Scand Suppl 176:90-5. 2000
    ..It remains to be proved that inhibition of the defective folding of amyloid-beta peptide and/or amyloid plaque deposition could be beneficial for the therapeutic treatment of Alzheimer's disease...
  9. ncbi request reprint The disulfide isomerase Grp58 is a protective factor against prion neurotoxicity
    Claudio Hetz
    Serono Pharmaceutical Research Institute, 1228 Plan les Ouates, Geneva, Switzerland
    J Neurosci 25:2793-802. 2005
    ..Our findings suggest that targeting Grp58 interaction may have applications for developing novel strategies for treatment and early diagnosis of prion diseases...
  10. ncbi request reprint Pharmacological profiles of peptide drug candidates for the treatment of Alzheimer's disease
    Celine Adessi
    Serono Pharmaceutical Research Institute, 1228 Geneva, Switzerland
    J Biol Chem 278:13905-11. 2003
    ..Our results suggest that the pharmacological profile of beta-sheet breaker peptides can be improved to produce compounds with drug-like properties that might offer a new promise in the treatment of Alzheimer's disease...
  11. pmc Prion replication alters the distribution of synaptophysin and caveolin 1 in neuronal lipid rafts
    Milene Russelakis-Carneiro
    Serono Pharmaceutical Research Institute, Geneva, Switzerland
    Am J Pathol 165:1839-48. 2004
    ..These results indicate that prion replication triggers an abnormal localization of caveolin 1 and synaptophysin, which in turn may alter neuronal function...
  12. pmc Prion protein misfolding affects calcium homeostasis and sensitizes cells to endoplasmic reticulum stress
    Mauricio Torres
    Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, Faculty of Medicine, University of Chile, Santiago, Chile
    PLoS ONE 5:e15658. 2010
    ..Our results suggest that alterations in calcium homeostasis and increased susceptibility to ER stress are common pathological features of both infectious and familial PrD models...
  13. pmc Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein
    Claudio Hetz
    Serono Pharmaceutical Research Institute, 14 Chemin des Aulx, 1228 Plan les Ouates, Switzerland
    EMBO J 22:5435-45. 2003
    ....
  14. ncbi request reprint Changes in the glycosylation pattern of prion protein in murine scrapie. Implications for the mechanism of neurodegeneration in prion diseases
    Milene Russelakis-Carneiro
    Serono Pharmaceutical Research Institute, 14, Chemin des Aulx, 1228 Geneva, Switzerland
    J Biol Chem 277:36872-7. 2002
    ..This is the first time that changes in the distribution and glycopattern of PrP have been described in an in vivo model of prion diseases...
  15. ncbi request reprint Is loss of function of the prion protein the cause of prion disorders?
    Claudio Hetz
    Serono Pharmaceutical Research Institute, 14 Chemin des Aulx, 1228 Plan les Ouates, Switzerland
    Trends Mol Med 9:237-43. 2003
    ..Thus, the mechanism of neurodegeneration in spongiform encephalopathies remains enigmatic...
  16. ncbi request reprint Reduction of amyloid load and cerebral damage in a transgenic mouse model of Alzheimer's disease by treatment with a beta-sheet breaker peptide
    Bruno Permanne
    Serono Pharmaceutical Research Institute, 1228 Geneva, Switzerland
    FASEB J 16:860-2. 2002
    ....
  17. ncbi request reprint A homogeneous 384-well high-throughput binding assay for a TNF receptor using alphascreen technology
    Janet Wilson
    Serono Pharmaceutical Research Institute, 14 ch des Aulx, 1228 Plan les Ouates, Geneva, Switzerland
    J Biomol Screen 8:522-32. 2003
    ..The results showed that the assay was able to detect such peptides and could be used to launch a high-throughput screening campaign for small molecules able to prevent OX40 receptor activation...
  18. doi request reprint Protein misfolding and neurodegeneration
    Claudio Soto
    Department of Neurology, Neuroscience and Cell Biology, University of Texas Medical Branch, 301 University Boulevard, Galveston, TX 77555, USA
    Arch Neurol 65:184-9. 2008
    ....
  19. pmc Unfolded protein response transcription factor XBP-1 does not influence prion replication or pathogenesis
    Claudio Hetz
    Department of Immunology and Infectious Diseases, Harvard School of Public Health, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 105:757-62. 2008
    ....
  20. pmc Perturbation of endoplasmic reticulum homeostasis facilitates prion replication
    Claudio Hetz
    Department of Neurology, University of Texas Medical Branch, Galveston, Texas 77555, USA
    J Biol Chem 282:12725-33. 2007
    ..Because PrP(Sc) induces ER stress, our data point to a vicious cycle accelerating prion replication, which may explain the rapid progression of the disease...
  21. pmc Crossing the species barrier by PrP(Sc) replication in vitro generates unique infectious prions
    Joaquin Castilla
    Department of Neurology, University of Texas Medical Branch, Galveston, TX 77555, USA
    Cell 134:757-68. 2008
    ..Our results indicate that PMCA is a valuable tool for the investigation of cross-species transmission and suggest that species barrier and strain generation are determined by the propagation of PrP misfolding...
  22. ncbi request reprint Isolation and characterization of a proteinase K-sensitive PrPSc fraction
    Miguel A Pastrana
    Prion Research Unit, Department of Medicine, School of Medicine, University of Santiago de Compostela, Rue de S Francisco s n, Santiago de Compostela, Galiza, Spain 15782
    Biochemistry 45:15710-7. 2006
    ..PK-sensitive PrPSc isolates should prove useful for structural studies to help understand fundamental issues of the molecular biology of PrPSc and in the quest to design tests to detect preclinical prion disease...
  23. ncbi request reprint Presymptomatic detection of prions in blood
    Paula Saa
    George and Cynthia Mitchell Center for Alzheimer s disease research, Department of Neurology, University of Texas Medical Branch, 301 University Boulevard, Galveston, TX 77555 0646, USA
    Science 313:92-4. 2006
    ..The ability to detect prions biochemically in the blood of infected but not clinically sick animals offers a great promise for the noninvasive early diagnosis of TSEs...
  24. ncbi request reprint Amyloids, prions and the inherent infectious nature of misfolded protein aggregates
    Claudio Soto
    George and Cynthia Mitchell Center for Alzheimer s disease and related Neurodegenerative Disorders, Departments of Neurology, University of Texas Medical Branch, Galveston, TX 77555, USA
    Trends Biochem Sci 31:150-5. 2006
    ..Intriguing recent data suggest that other protein misfolding disorders might also be transmitted by a prion-like infectious process...
  25. pmc Stressing out the ER: a role of the unfolded protein response in prion-related disorders
    Claudio A Hetz
    Harvard School of Public Health, Department of Immunology and Infectious Diseases, Boston, MA 02115, USA
    Curr Mol Med 6:37-43. 2006
    ..These findings have applications for developing novel strategies for treatment and early diagnosis of transmissible spongiform encephalopathies and other neurodegenerative diseases...
  26. ncbi request reprint Detection of prions in blood
    Joaquin Castilla
    Department of Neurology, University of Texas Medical Branch, 301 University Boulevard, Galveston, Texas, 77555 0646, USA
    Nat Med 11:982-5. 2005
    ..These findings represent the first time that PrP(Sc) has been detected biochemically in blood, offering promise for developing a noninvasive method for early diagnosis of prion diseases...
  27. ncbi request reprint Molecular mechanisms of neurotoxicity of pathological prion protein
    Joaquin Castilla
    University of Texas Medical Branch, Galveston, TX 77555, USA
    Curr Mol Med 4:397-403. 2004
    ....
  28. pmc A novel human disease with abnormal prion protein sensitive to protease
    Pierluigi Gambetti
    Institute of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    Ann Neurol 63:697-708. 2008
    ....
  29. ncbi request reprint The controversial protein-only hypothesis of prion propagation
    Claudio Soto
    Department of Neurology, University of Texas Medical Branch, Galveston, Texas, USA
    Nat Med 10:S63-7. 2004
    ..The evidence in favor of the prion model is very strong, but final proof-consisting of the generation of infectious prions in vitro-is still missing...
  30. pmc Accelerated high fidelity prion amplification within and across prion species barriers
    Kristi M Green
    Department of Microbiology, Immunology and Molecular Genetics, University of Kentucky, Lexington, Kentucky, United States of America
    PLoS Pathog 4:e1000139. 2008
    ..Our studies demonstrate that combined transgenic mouse and PMCA approaches not only expedite intra- and inter-species prion transmission, but also provide a facile means of generating and characterizing novel prion strains...
  31. ncbi request reprint Passage of murine scrapie prion protein across the mouse vascular blood-brain barrier
    William A Banks
    GRECC, Veterans Affairs Medical Center St Louis and Division of Geriatrics, Department of Internal Medicine, Saint Louis University School of Medicine, 915 N Grand Blvd, St Louis, MO 63106, USA
    Biochem Biophys Res Commun 318:125-30. 2004
    ..PrP(SC) also entered the cerebrospinal fluid (CSF) compartment. These results show that a prion protein can cross the intact BBB to enter both the parenchymal and CSF compartments of the brain...
  32. pmc Cell-free propagation of prion strains
    Joaquin Castilla
    Department of Neurology, University of Texas Medical Branch, Galveston, TX 77555, USA
    EMBO J 27:2557-66. 2008
    ..These results provide additional support for the prion hypothesis and indicate that strain characteristics can be faithfully propagated in the absence of living cells, suggesting that strain variation is dependent on PrP(Sc) properties...
  33. pmc Endoplasmic reticulum stress, PrP trafficking, and neurodegeneration
    Claudio Soto
    George and Cynthia Mitchell Center for Neurodegenerative Diseases, Department of Neurology, University of Texas Medical Branch, Galveston, TX 77555, USA
    Dev Cell 15:339-41. 2008
    ..This pathway puts together several pieces in the puzzle of the relationship between PrP(Sc) and brain damage and may in part explain the mechanism of prion neurodegeneration...
  34. ncbi request reprint Emerging roles of the unfolded protein response signaling in physiology and disease
    Claudio A Hetz
    Curr Mol Med 6:1. 2006
  35. pmc Reversibility of prion-induced neurodegeneration
    Claudio Soto
    Lancet Neurol 6:294-5. 2007
  36. ncbi request reprint Pre-symptomatic detection of prions by cyclic amplification of protein misfolding
    Claudio Soto
    Department of Neurology, University of Texas Medical Branch, Galveston, TX 77555, USA
    FEBS Lett 579:638-42. 2005
    ..Our findings indicate that PMCA may be useful for the development of an ultra-sensitive diagnostic test to minimize the risk of further propagation of TSEs...
  37. pmc The prion strain phenomenon: molecular basis and unprecedented features
    Rodrigo Morales
    Protein Misfolding Disorders Laboratory, George and Cynthia Mitchell Center for Neurodegenerative Diseases, Department of Neurology, University of Texas Medical Branch, Galveston, TX 77555 0646, USA
    Biochim Biophys Acta 1772:681-91. 2007
    ..The dynamic nature and inter-relations between strains and the potential for the generation of a large number of new prion strains is the perfect recipe for the emergence of extremely dangerous new infectious agents...
  38. pmc Production of cattle lacking prion protein
    Jürgen A Richt
    National Animal Disease Center, Agriculture Research Services, United States Department of Agriculture, 2300 Dayton Avenue, Ames, Iowa 50010, USA
    Nat Biotechnol 25:132-8. 2007
    ..PrP(C)-deficient cattle may be a useful model for prion research and could provide industrial bovine products free of prion proteins...
  39. ncbi request reprint In vitro generation of infectious scrapie prions
    Joaquin Castilla
    Department of Neurology, University of Texas Medical Branch, Galveston, Texas 77555, USA
    Cell 121:195-206. 2005
    ..These findings demonstrate that prions can be generated in vitro and provide strong evidence in support of the protein-only hypothesis of prion transmission...
  40. ncbi request reprint Protein misfolding cyclic amplification for diagnosis and prion propagation studies
    Joaquin Castilla
    Univeristy of Texas, Medical Branch, Galveston, 77555, USA
    Methods Enzymol 412:3-21. 2006
    ..We also describe in detail the technical and methodological aspects of PMCA, as well as its application in automatic and serial modes that have been developed with a view to improving disease diagnosis...
  41. ncbi request reprint Ultra-efficient replication of infectious prions by automated protein misfolding cyclic amplification
    Paula Saa
    George and Cynthia Mitchell Center for Alzheimer Disease and Related Neurodegenerative Disorders, Department of Neurology, University of Texas Medical Branch, Galveston, Texas 77555, USA
    J Biol Chem 281:35245-52. 2006
    ..Therefore, PMCA offers great promise for the development of highly sensitive, specific, and early diagnosis of transmissible spongiform encephalopathy and to further understand the molecular basis of prion propagation...
  42. ncbi request reprint Protein misfolding disorders and rational design of antimisfolding agents
    Lisbell D Estrada
    Protein Misfolding Disorders Laboratory, Department of Neurology, University of Texas Medical Branch, Galveston, TX, USA
    Methods Mol Biol 340:277-93. 2006
    ..In this chapter, we survey the mechanism of protein misfolding and some strategies to rationally produce inhibitors of this process...
  43. ncbi request reprint Cyclic amplification of protein misfolding and aggregation
    Paula Saa
    Department of Neurology, University of Texas Medical Branch, Galveston, TX, USA
    Methods Mol Biol 299:53-65. 2005
    ....
  44. pmc Detection of infectious prions in urine
    Dennisse Gonzalez-Romero
    George and Cynthia Mitchell Center for Neurodegenerative Diseases, Departments of Neurology, Neuroscience and Cell Biology and Biochemistry and Molecular Biology, University of Texas Medical Branch, 301 University Boulevard, Galveston, TX 77555 0646, USA
    FEBS Lett 582:3161-6. 2008
    ..Our data indicate that low quantities of infectious prions are excreted in the urine. These findings suggest that urine is a possible source of prion transmission...