Claudio Soto

Summary

Affiliation: University of Texas Medical Branch
Country: USA

Publications

  1. ncbi 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
  2. ncbi 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
  3. ncbi 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
  4. doi 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
  5. ncbi 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
  6. 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
  7. pmc Molecular cross talk between misfolded proteins in animal models of Alzheimer's and prion diseases
    Rodrigo Morales
    Protein Misfolding Disorders Laboratory, Department of Neurology, Mitchell Center for Alzheimer s Disease and Related Brain Disorders, University of Texas Medical School at Houston, Houston, Texas 77030, USA
    J Neurosci 30:4528-35. 2010
  8. pmc De novo generation of infectious prions in vitro produces a new disease phenotype
    Marcelo A Barria
    George and Cynthia Mitchell Center for Neurodegenerative Diseases, University of Texas Medical Branch, Galveston, TX, USA
    PLoS Pathog 5:e1000421. 2009
  9. 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
  10. pmc Protein misfolding cyclic amplification of infectious prions
    Rodrigo Morales
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas, USA
    Nat Protoc 7:1397-409. 2012

Collaborators

Detail Information

Publications63

  1. ncbi 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...
  2. ncbi 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...
  3. ncbi 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...
  4. doi 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
    ....
  5. ncbi 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...
  6. 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...
  7. pmc Molecular cross talk between misfolded proteins in animal models of Alzheimer's and prion diseases
    Rodrigo Morales
    Protein Misfolding Disorders Laboratory, Department of Neurology, Mitchell Center for Alzheimer s Disease and Related Brain Disorders, University of Texas Medical School at Houston, Houston, Texas 77030, USA
    J Neurosci 30:4528-35. 2010
    ..Our findings may have important implications to understand the origin and progression of PMDs...
  8. pmc De novo generation of infectious prions in vitro produces a new disease phenotype
    Marcelo A Barria
    George and Cynthia Mitchell Center for Neurodegenerative Diseases, University of Texas Medical Branch, Galveston, TX, USA
    PLoS Pathog 5:e1000421. 2009
    ..The findings also suggest that prion diversity is not restricted to those currently known, and that likely new forms of infectious protein foldings may be produced, resulting in novel disease phenotypes...
  9. 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...
  10. pmc Protein misfolding cyclic amplification of infectious prions
    Rodrigo Morales
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas, USA
    Nat Protoc 7:1397-409. 2012
    ....
  11. pmc Misfolded protein aggregates: mechanisms, structures and potential for disease transmission
    Ines Moreno-Gonzalez
    Mitchell Center Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, 6431 Fannin St, Houston, TX 77030, USA
    Semin Cell Dev Biol 22:482-7. 2011
    ..Future research in this field should aim to clarify this possibility and translate the knowledge of the basic disease mechanisms into development of novel strategies for early diagnosis and efficient treatment...
  12. ncbi 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...
  13. ncbi 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...
  14. pmc Cyclic amplification of prion protein misfolding
    Marcelo A Barria
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, TX, USA
    Methods Mol Biol 849:199-212. 2012
    ..In this chapter, we describe a detailed protocol of the PMCA technique, highlighting some of the important technical aspects to obtain a successful and reproducible application of the technology...
  15. ncbi Natural animal models of neurodegenerative protein misfolding diseases
    Ines Moreno-Gonzalez
    University of Texas Medical School at Houston, Houston, Texas, 77030, USA
    Curr Pharm Des 18:1148-58. 2012
    ..Although the usefulness of transgenic mice is unquestionable, the information obtained from natural non-transgenic models could be very valuable to fully understand the pathogenesis of these devastating diseases...
  16. pmc Estimating prion concentration in fluids and tissues by quantitative PMCA
    Baian Chen
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas, USA
    Nat Methods 7:519-20. 2010
    ..Using this procedure, we determined the quantities of misfolded prion protein (PrP(Sc)) in brain, spleen, blood and urine of scrapie-affected hamsters...
  17. pmc Generation of prions in vitro and the protein-only hypothesis
    Rodrigo Diaz-Espinoza
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, The University of Texas Health Science Center, Houston, TX, USA
    Prion 4:53-9. 2010
    ..In this article, we discuss these recent reports under the context of the protein-only hypothesis and their implications...
  18. 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...
  19. 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...
  20. 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...
  21. ncbi 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...
  22. ncbi 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...
  23. ncbi Smoking exacerbates amyloid pathology in a mouse model of Alzheimer's disease
    Ines Moreno-Gonzalez
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Medical School at Houston, Houston, Texas, USA
    Nat Commun 4:1495. 2013
    ..Our findings suggest that cigarette smoking may increase Alzheimer's disease onset and exacerbate its features and thus, may constitute an important environmental risk factor for Alzheimer's disease...
  24. ncbi 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...
  25. pmc Kosmotropic anions promote conversion of recombinant prion protein into a PrPSc-like misfolded form
    Rodrigo Diaz-Espinoza
    Department of Neurology, Mitchell Center for Alzheimer s Disease and Related Brain Disorders, University of Texas Houston Medical School, Houston, Texas, United States of America
    PLoS ONE 7:e31678. 2012
    ....
  26. 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...
  27. pmc Computational selection of inhibitors of Abeta aggregation and neuronal toxicity
    Deliang Chen
    Sealy Center for Structural Biology and Molecular Biophysics, Department of Biochemistry and Molecular Biology, UTMB, Galveston, TX 77555 0857, USA
    Bioorg Med Chem 17:5189-97. 2009
    ..They also prevented the toxic effects of aggregated Abeta on neuroblastoma cells. Their low molecular weight and aqueous solubility makes them promising lead compounds for treating AD...
  28. ncbi 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
    ....
  29. pmc In vivo generation of neurotoxic prion protein: role for hsp70 in accumulation of misfolded isoforms
    Pedro Fernandez-Funez
    Department of Neurology, University of Texas Medical Branch, Galveston, TX, USA
    PLoS Genet 5:e1000507. 2009
    ..Overall, we provide new insight into the mechanisms of spontaneous accumulation of neurotoxic PrP and uncover the potential therapeutic role of Hsp70 in treating these devastating disorders...
  30. pmc Reduction of prion infectivity in packed red blood cells
    Rodrigo Morales
    Department of Neurology, University of Texas Medical Branch, 301 University Boulevard, Galveston, TX 77555, USA
    Biochem Biophys Res Commun 377:373-8. 2008
    ..This technology may lead to increased safety of blood products and reduce the risk of further propagation of prion diseases...
  31. pmc Calcineurin inhibition at the clinical phase of prion disease reduces neurodegeneration, improves behavioral alterations and increases animal survival
    Abhisek Mukherjee
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas, United States of America
    PLoS Pathog 6:e1001138. 2010
    ..Our findings contribute to an understanding of the mechanism of neurodegeneration in prion diseases and more importantly may provide a novel strategy for therapy that is beneficial at the clinical phase of the disease...
  32. ncbi 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
    ....
  33. pmc High-resolution structure of infectious prion protein: the final frontier
    Rodrigo Diaz-Espinoza
    Department of Neurology, Mitchell Center for Alzheimer s Disease and Related Brain Disorders, University of Texas Medical School, Houston, Texas, USA
    Nat Struct Mol Biol 19:370-7. 2012
    ..This review highlights the importance of, challenges for and recent progress toward elucidating the elusive structure of PrP(Sc), arguably the major pending milestone to reach in understanding prions...
  34. ncbi 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...
  35. pmc Cellular factors implicated in prion replication
    Karim Abid
    Department of Neurology, University of Texas Medical Branch, Galveston, TX, USA
    FEBS Lett 584:2409-14. 2010
    ..More research is needed to elucidate the identity of these factors, their detailed mechanism of action and whether or not they are essential component of the infectious particle...
  36. pmc Initial fate of prions upon peripheral infection: half-life, distribution, clearance, and tissue uptake
    Akihiko Urayama
    Department of Neurology, The University of Texas Medical School at Houston, Houston, Texas 77030, USA
    FASEB J 25:2792-803. 2011
    ..Our results provide a fundamental pharmacokinetic characterization of PrP(Sc) in vivo, which may be relevant to estimate tissue risks and mechanisms of prion neuroinvasion and to identify novel therapeutic strategies...
  37. pmc Generation of a new form of human PrP(Sc) in vitro by interspecies transmission from cervid prions
    Marcelo A Barria
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Medical School at Houston, Houston, Texas 77030, USA
    J Biol Chem 286:7490-5. 2011
    ..If our findings are corroborated by infectivity assays, they will imply that CWD prions have the potential to infect humans and that this ability progressively increases with CWD spreading...
  38. pmc Aggregate-depleted brain fails to induce Aβ deposition in a mouse model of Alzheimer's disease
    Claudia Duran-Aniotz
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas, United States of America Universidad de los Andes, Facultad de Medicina, Santiago, Chile
    PLoS ONE 9:e89014. 2014
    ..These findings shed light respect to the active principle responsible for the prion-like spreading of Alzheimer's amyloid pathology and open the possibility of using seeds-capturing reagents as a promising target for AD treatment. ..
  39. pmc Cross currents in protein misfolding disorders: interactions and therapy
    Rodrigo Morales
    Protein Misfolding Disorders Laboratory, Department of Neurology, University of Texas Medical School at Houston, 6431 Fannin Street, Houston, Texas 77030, USA
    CNS Neurol Disord Drug Targets 8:363-71. 2009
    ..Due to their similar molecular mechanisms, common therapeutic strategies could be applied for the diseases in this group...
  40. ncbi Amyloid inhibitors and beta-sheet breakers
    Claudio Soto
    Protein Misfolding Disorders Laboratory, Department of Neurology, University of Texas Medical Branch, Galveston, TX, USA
    Subcell Biochem 38:351-64. 2005
    ..In this article we review this approach and summarize the data supporting the view that beta-sheet breakers could be serious candidates to combat this devastating disease...
  41. pmc Prion hypothesis: the end of the controversy?
    Claudio Soto
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, The University of Texas Medical School at Houston, 6431 Fannin St, Houston, TX 77030, USA
    Trends Biochem Sci 36:151-8. 2011
    ..Future research promises to reveal many more intriguing features about the rogue prions...
  42. ncbi Amyloid formation modulates the biological activity of a bacterial protein
    Sylvain Bieler
    Department of Neurology, University of Texas Medical Branch, Galveston, Texas 77555, USA
    J Biol Chem 280:26880-5. 2005
    ....
  43. pmc Microcin amyloid fibrils A are reservoir of toxic oligomeric species
    Mohammad Shahnawaz
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas 77030, USA
    J Biol Chem 287:11665-76. 2012
    ..These findings may have substantial implications to understand the dynamic process of interconversion between toxic and non-toxic aggregated species implicated in protein misfolding diseases...
  44. ncbi Detection of misfolded Aβ oligomers for sensitive biochemical diagnosis of Alzheimer's disease
    Natalia Salvadores
    Mitchell Center for Alzheimer s Disease and Related Brain Disorders, Department of Neurology, University of Texas Medical School at Houston, 6431 Fannin Street, Houston, TX 77030, USA
    Cell Rep 7:261-8. 2014
    ..These findings provide the proof-of-principle basis for developing a highly sensitive and specific biochemical test for AD diagnosis. ..
  45. ncbi 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
    ....
  46. ncbi 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...
  47. ncbi 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...
  48. ncbi 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...
  49. pmc Reversibility of prion-induced neurodegeneration
    Claudio Soto
    Lancet Neurol 6:294-5. 2007
  50. 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...
  51. 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
    ....
  52. ncbi 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...
  53. 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
    ....
  54. 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...
  55. ncbi 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...
  56. ncbi 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
  57. ncbi 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...
  58. pmc Bactericidal activity of both secreted and nonsecreted microcin E492 requires the mannose permease
    Sylvain Bieler
    Department of Pathology and Immunology, University of Geneva, 1 rue Michel Servet, 1211 Geneva 4, Switzerland
    J Bacteriol 188:7049-61. 2006
    ..Based on these findings, we propose a new model for cell entry, inner membrane insertion, and toxic activity of MccE492...
  59. 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...
  60. 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
    ....
  61. 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...
  62. ncbi Emerging roles of the unfolded protein response signaling in physiology and disease
    Claudio A Hetz
    Curr Mol Med 6:1. 2006
  63. ncbi 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...

Research Grants5

  1. Neurodegeneration in prion diseases: Therapy and diagnosis
    Claudio Soto; Fiscal Year: 2007
    ..The findings generated in this project will contribute to understand the molecular basis of prion propagation, neuronal death and brain damage in TSE and will provide novel targets for therapy and diagnosis of these diseases. ..
  2. Small molecule Beta-sheet breaker peptidemimetics for Alzheimer's therapy
    Claudio Soto; Fiscal Year: 2007
    ..The findings obtained in this project should lead to the generation of second generation non-peptide beta-sheet breakers with improved drug-like properties, which will offer a great promise for the efficient treatment of AD. ..