Research Topics
Genomes and GenesSpecies | Paul BrownSummaryAffiliation: National Institutes of Health Country: USA Publications
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Publications
New studies on the heat resistance of hamster-adapted scrapie agent: threshold survival after ashing at 600 degrees C suggests an inorganic template of replicationP Brown
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
Proc Natl Acad Sci U S A 97:3418-21. 2000..These results suggest that an inorganic molecular template with a decomposition point near 600 degrees C is capable of nucleating the biological replication of the scrapie agent...
Ultra-high-pressure inactivation of prion infectivity in processed meat: a practical method to prevent human infectionPaul Brown
National Institutes of Health, Bethesda, MD 20892, USA
Proc Natl Acad Sci U S A 100:6093-7. 2003....
Drug therapy in human and experimental transmissible spongiform encephalopathyPaul Brown
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 4122, USA
Neurology 58:1720-5. 2002..Also, very recent work in developing antibodies that can neutralize in vitro infection (and, in conjunction with genetic engineering, in vivo infection) has renewed interest in the strategies of both active and passive immunization...
The pathogenesis of transmissible spongiform encephalopathy: routes to the brain and the erection of therapeutic barricadesP Brown
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892 4122, USA
Cell Mol Life Sci 58:259-65. 2001..Possible modes and sites of therapeutic intervention are suggested...
Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution, and current concernsP Brown
National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA
Emerg Infect Dis 7:6-16. 2001..Government agencies in many countries continue to implement new measures to minimize this risk...
Iatrogenic Creutzfeldt-Jakob disease at the millenniumP Brown
Laboratory of CNS Studies, NINDS, NIH, Bethesda, MD 20892, USA
Neurology 55:1075-81. 2000....
Fall-out from a possible transfusion-related transmission of vCJDPaul Brown
National Institutes of Health, Bethesda, Maryland, USA
Lancet Neurol 3:203. 2004
The modern landscape of transfusion-related iatrogenic Creutzfeldt-Jakob disease and blood screening testsPaul Brown
National Institutes of Health, Bethesda, Maryland, USA
Curr Opin Hematol 11:351-6. 2004....
Working with transmissible spongiform encephalopathy agentsPaul Brown
National Institutes of Health, Bethesda, Maryland, USA
ILAR J 46:44-52. 2005..In this review, precautions and regulations concerning the handling of TSE agents are discussed in relation to personnel and environmental biosafety...
Infectivity studies of both ash and air emissions from simulated incineration of scrapie-contaminated tissuesPaul Brown
Laboratory of CNS Studies, National Institute of Neurological Disorders and Stroke, and Div of Environmental Protection, Office of Research Facilities Development and Operations, NIH, US Dept of HHS, Bethesda, MD 20892, USA
Environ Sci Technol 38:6155-60. 2004..The extent to which this result can be realized in actual incinerators and other combustion devices will depend on equipment design and operating conditions during the heating process...
Long-term mortality in the United States cohort of pituitary-derived growth hormone recipientsJames L Mills
National Institute of Child Health and Human Development, National Institutes of Health, Department of Health and Human Services, 6100 Building Room 7B03, Bethesda, MD 20892, USA
J Pediatr 144:430-6. 2004..We investigated whether they were at increased risk of death from other conditions, particularly preventable conditions...
Spontaneous mutations in the prion protein gene causing transmissible spongiform encephalopathyAyush Dagvadorj
National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
Ann Neurol 52:355-9. 2002..We provide evidence that hereditary and apparently sporadic transmissible spongiform encephalopathy cases associated with the D178N mutation result from multiple recurrent mutational events...
How do neurons degenerate in prion diseases or transmissible spongiform encephalopathies (TSEs): neuronal autophagy revisitedPaweł P Liberski
Laboratory of Electron Microscopy and Neuropathology, Department of Molecular Biology, Medical Academy Łódź, Poland
Acta Neurobiol Exp (Wars) 62:141-7. 2002..Finally, a large area of the cytoplasm was transformed into a collection of autophagic vacuoles of different sizes. Virtually identical alterations, albeit with much lower frequency, were seen in terminally ill CJD-affected hamsters...
Pathological prion protein in muscles of hamsters and mice infected with rodent-adapted BSE or vCJDAchim Thomzig
Robert Koch-Institut (P24 - Transmissible Spongiform Encephalopathies, Berlin, Germany
J Gen Virol 87:251-4. 2006..Our findings emphasize the need for further assessment of possible public-health risks from TSE involvement of skeletal muscle...
Advances in screening test development for transmissible spongiform encephalopathiesLarisa Cervenakova
American Red Cross Research and Development, 15601 Crabbs Branch Way, Rockville, MD 20855, USA
Expert Rev Anti Infect Ther 2:873-80. 2004..However, the quest for a blood test is still in its infancy and requires extensive further research...
Inactivation of transmissible spongiform encephalopathy agents in food products by ultra high pressure-temperature treatmentFranco Cardone
Degenerative and Inflammatory Neurological Diseases Unit, Department of Cell Biology and Neurosciences, Istituto Superiore di Sanita, Viale Regina Elena 299, 00161 Rome, Italy
Biochim Biophys Acta 1764:558-62. 2006..These data indicate that the high pressure-temperature treatment is a ready-to-use and feasible strategy to reduce the risk of TSEs transmission via contaminated meat products...
Novel methods for disinfection of prion-contaminated medical devicesGuillaume Fichet
CEA/DSV/DRM/GIDTIP, Route du Panorama, 92265 Fontenay-aux-Roses, France
Lancet 364:521-6. 2004..INTERPRETATION: New decontamination procedures are proposed to ensure the safety of medical and surgical instruments as well as surfaces that cannot withstand the currently recommended prion inactivation procedures...
Exuberant cellular reaction of the optic nerves in experimental Creutzfeldt-Jakob diseasePaweł P Liberski
Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Poland
Acta Neurobiol Exp (Wars) 63:309-18. 2003..An analogous network of narrow cisterns was seen to surround whole segments of the myelinated fibers...
Tubulovesicular structures are a consistent (and unexplained) finding in the brains of humans with prion diseasesPawel P Liberski
Department of Molecular Pathology and Neuropathology, Medical University of Lodz, PL 92 216 Lodz, Poland
Virus Res 132:226-8. 2008..This study confirms the TSE-specificity of TVS, the morphology of which suggests a possible pathogenetic role and relationship to recently described virion-like arrays of 25nm particles in scrapie-infected tissue cultures...
Variant CJD transmission through blood: risks to predictors and "predictees"Paul Brown
Transfusion 43:425-7. 2003
Transmissible spongiform encephalopathy update and implications for blood safetyMaura N Ricketts
World Health Organization, Room L412, Ave Appia, Geneva, Switzerland
Clin Lab Med 23:129-37. 2003..Surveillance of human TSEs and investigation of the risk of transfusion transmission must continue in order to provide further refinements in blood safety policy...
Astrocytes in transmissible spongiform encephalopathies (prion diseases)Paweł P Liberski
Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Lodz, Poland
Folia Neuropathol 42:71-88. 2004..An interesting interaction between astrocytes and oligodendrocytes is discussed in detail as well as a particular form of astrocytic reaction in panencephalopathic form of TSEs...
Iatrogenic Creutzfeldt-Jakob disease: the waning of an eraPaul Brown
Neurology 67:389-93. 2006..Therefore, at least in those countries in which variant CJD has occurred, precautionary measures must continue for the indefinite future...
Infectious agent of sheep scrapie may persist in the environment for at least 16 yearsGudmundur Georgsson
Institute for Experimental Pathology, University of Iceland, Keldur v Vesturlandsveg, IS 112 Reykjavik, Iceland
J Gen Virol 87:3737-40. 2006..Epidemiological investigation established with near certitude that the disease had not been introduced from the outside and it is concluded that the agent may have persisted in the old sheep-house for at least 16 years...
Subependymal plaques in scrapie-affected hamster brains--why are they so different from compact kuru plaques?Beata Sikorska
Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Czechoslowacka Street 8 10, PL 92 216 Lodz, Poland
Folia Neuropathol 46:32-42. 2008..Some microglial cells were observed in close contact with PrP-positive plaques, and secondary lysosomes within these cells were heavily decorated with gold particles...
Clinical, neuropathological and immunohistochemical features of sporadic and variant forms of Creutzfeldt-Jakob disease in the squirrel monkey (Saimiri sciureus)Lawrence Williams
Department of Veterinary Sciences, Michale E Keeling Center for Comparative Medicine and Research, University of Texas MD Anderson Cancer Center, Bastrop, TX, USA
J Gen Virol 88:688-95. 2007..Human strains of sCJD and vCJD cause distinguishable clinicopathological features in the squirrel monkey that can provide a baseline for the evaluation of future therapeutic studies...
Role of a medical social worker in improving quality of life for patients with advanced cancer with a structured multidisciplinary interventionJanis J Miller
Medial Social Services, Mayo Clinic, Rochester, MN 55905, USA
J Psychosoc Oncol 25:105-19. 2007..Outlined in this paper is the role of the medical social worker in a clinical trial, how education was provided and strategies for future interventions. doi:10.1300/J077v25n04_07...
Comparison of CR36, a new heparan mimetic, and pentosan polysulfate in the treatment of prion diseasesClaire Larramendy Gozalo
CEA, IMETI SEPIA, 18 route du Panorama, BP6, 92265 Fontenay aux Roses Cedex, France
J Gen Virol 88:1062-7. 2007..These results show, once again, that anti-TSE drugs cannot be encouraged for human therapeutic trials solely on the basis of in vitro or ex vivo observations, but must first be subjected to in vivo animal studies...
Cell death and autophagy in prion diseases (transmissible spongiform encephalopathies)Paweł P Liberski
Department of Molecular Pathology and Neuropathology, Chair of Oncology, Medical University of Lodz, Czechosłowacka Str 8 10, PL 92 216 Lodz, Poland
Folia Neuropathol 46:1-25. 2008..On the basis of ultrastructural studies, we suggest that autophagy may play a major role in transmissible spongiform encephalopathies and may even participate in the formation of spongiform change...
On the question of sporadic or atypical bovine spongiform encephalopathy and Creutzfeldt-Jakob diseasePaul Brown
Emerg Infect Dis 12:1816-21. 2006....
Trends in admissions to hospital involving an assault using a knife or other sharp instrument, England, 1997-2005Roy Maxwell
Department of Social Medicine, University of Bristol, Canynge Hall, Bristol BS8 2PR, UK
J Public Health (Oxf) 29:186-90. 2007..Main outcome measure All completed hospital admissions with a mention of assault by sharp object (International Classification of Diseases, Tenth Revision, code X99) in the relevant diagnosis fields in HES...
Transmissible spongiform encephalopathy in the 21st century: neuroscience for the clinical neurologistPaul Brown
Neurology 70:713-22. 2008
Divergent expression of cellular prion protein on blood cells of human and nonhuman primatesKarel Holada
Department of Immunology and Microbiology, 1st Medical Faculty, Charles University, Prague, Czech Republic
Transfusion 47:2223-32. 2007..Nonhuman primates are the most relevant models of human prion diseases...
Neuronal and astrocytic cells, obtained after differentiation of human neural GFAP-positive progenitors, present heterogeneous expression of PrPcMonika Witusik
Department of Molecular Pathology and Neuropathology, Chair of Oncology, Medical University of Lodz, 8 10 Czechoslowacka str, Lodz, Poland
Brain Res 1186:65-73. 2007..Of note, glial and neuronal cells showed a very large heterogeneity of PrP(c) expression. Our results provide the basis for studying the role of PrP(c) in cell differentiation and neurogenesis from human GFAP-positive progenitor cells...
Neuronal autophagy and aggresomes constitute a consistent part of neurodegeneration in experimental scrapieBeata Sikorska
Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Czechoslowacka Street 8 10, PL 92 216 Lodz, Poland
Folia Neuropathol 45:170-8. 2007..In a few specimens there were round electron-dense structures that we identified as aggresomes. Aggresomes are not membrane-bound and were found in the cytoplasm of a few neurons...
Detection of misfolded prion protein in blood with conformationally sensitive peptidesTao Pan
Adlyfe Inc, 9430 Key West Avenue, Rockville, MD 20850, USA
Transfusion 47:1418-25. 2007....
Will improvement in quality of life (QOL) impact fatigue in patients receiving radiation therapy for advanced cancer?Paul Brown
Department of Oncology, Mayo Clinic Rochester, Rochester, MN 55905, USA
Am J Clin Oncol 29:52-8. 2006..In this project, we examined the impact that participation in a randomized controlled trial of a multidisciplinary intervention designed to impact overall QOL had on fatigue for advanced cancer patients actively receiving treatment...
Ultrastructural studies of experimental scrapie and Creutzfeldt-Jakob disease in hamsters. I. Alterations of myelinated axonsPaweł P Liberski
Laboratory of Electron Microscopy and Neuropathology, Department of Molecular Biology, Medical Academy Łódź, Poland
Acta Neurobiol Exp (Wars) 62:121-9. 2002..In contrast, mice infected with the panencephalopathic Fujisaki strain of CJD exhibited much more elaborate changes of myelinated fibres...
Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UKJean Philippe Brandel
National Reference Centre of Iatrogenic CJD, Salpetriere Hospital, 75651 Cedex 13, Paris, France
Lancet 362:128-30. 2003..There is no evident explanation for this different distribution, which might be due to infection with different strains of prion in human growth hormone...
Temozolomide: too early for definitive conclusionsPaul Brown
J Clin Oncol 21:3710. 2003
Similar levels of infectivity in the blood of mice infected with human-derived vCJD and GSS strains of transmissible spongiform encephalopathyLarisa Cervenakova
Jerome H Holland Laboratory for the Biomedical Sciences, Red Cross, Rockville, MD 20855, USA
Transfusion 43:1687-94. 2003....
Partitioning of human and sheep forms of the pathogenic prion protein during the purification of therapeutic proteins from human plasmaChristopher J Stenland
Department of Pathogen Safety Research, Bayer Biological Products, Research Triangle Park, North Carolina 27709, USA
Transfusion 42:1497-500. 2002..Sheep scrapie (PrP(Sc)) was similarly evaluated...
Qualifications to the report of a new case of Creutzfeldt-Jakob disease in the recipient of a corneal transplantR Nick Hogan
Dallas, Tex
Arch Neurol 60:293-4; author reply 294. 2003
Fate of myelinated fibres in the optic nerves in experimental Creutzfeldt-Jakob disease in rodents: an ultrastructural studyBeata Sikorska
Department of Molecular Pathology and Neuropathology, Chair of Oncology, , , Poland
Folia Neuropathol 42:101-5. 2004..This finding suggests that myelin lamellae participate in the formation of vacuoles...
Neuronal cell death in transmissible spongiform encephalopathies (prion diseases) revisited: from apoptosis to autophagyPawel P Liberski
Department of Molecular Pathology and Neuropathology, Medical University Lodz, Czechoslowacka Street 8 10 pl 92 216 Lodz, Poland
Int J Biochem Cell Biol 36:2473-90. 2004..On a basis of ultrastructural studies, we suggest that autophagy plays a major role in transmissible spongiform encephalopathies (TSEs) and may even participate in a formation of spongiform change...
Autophagy is a part of ultrastructural synaptic pathology in Creutzfeldt-Jakob disease: a brain biopsy studyBeata Sikorska
Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Lodz, Poland
Int J Biochem Cell Biol 36:2563-73. 2004..Autophagic vacuoles are formed in many synapses in all categories of human transmissible encephalopathies. We conclude that synaptic autophagy contributes to overall synaptic loss in brains affected in prion diseases...
Ultrastructural studies of experimental scrapie and Creutzfeldt-Jakob disease in hamsters. II. Astrocytic and macrophage reaction towards axonal destructionPaweł P Liberski
Laboratory of Electron Microscopy and Neuropathology, Department of Molecular Biology, Medical Academy Łódź, Poland
Acta Neurobiol Exp (Wars) 62:131-9. 2002..Several mylinated fibres were clearly engulfed by the cytoplasm of a macrophage containing unusual annulate lamellae...
A prion lexicon (out of control)Paul Brown
Lancet 365:122. 2005
BSE infection of the small short-lived primate Microcebus murinusNoëlle Bons
Laboratoire de Neuromorphologie Fonctionnelle, EPHE, Universite Montpellier II, 34095 Montpellier, France
C R Biol 325:67-74. 2002....
Effect of protease treatment on plasma infectivity in variant Creutzfeldt-Jakob disease miceOksana Yakovleva
Jerome H. Holland Laboratory for the Biomedical Sciences, American Red Cross, Rockville, Maryland 20855, USA
Transfusion 44:1700-5. 2004..It is concluded that the use of PK in protocols for the detection of PrPres may decrease the sensitivity of blood-based assays...
Removal of exogenous (spiked) and endogenous prion infectivity from red cells with a new prototype of leukoreduction filterSamuel Sowemimo-Coker
Pall Medical, Pall Corp, Port Washington, New York 11050, USA
Transfusion 45:1839-44. 2005..In the absence of a preclinical screening test, removal of the infectious agent by processing is the only means by which risk to recipients of blood from donors with inapparent vCJD infections can be eliminated...
Impacting quality of life for patients with advanced cancer with a structured multidisciplinary intervention: a randomized controlled trialTeresa A Rummans
Department of Psychiatry and Psychology, Mayo Clinic Cancer Center, and Medical Social Services, Mayo Clinic, Rochester MN 55905, USA
J Clin Oncol 24:635-42. 2006....
Failure of immunocompetitive capillary electrophoresis assay to detect disease-specific prion protein in buffy coat from humans and chimpanzees with Creutzfeldt-Jakob diseaseLarisa Cervenakova
Plasma Derivatives Department, Jerome H Holland Laboratory for the Biomedical Sciences, American Red Cross, Rockville, MD 20855, USA
Electrophoresis 24:853-9. 2003..Thus, the ICCE assay as presently performed is not suitable for use as a screening test in human transmissible spongiform encephalopathies (TSEs)...
