Quantification of surviving cerebellar granule neurones and abnormal prion protein (PrPSc) deposition in sporadic Creutzfeldt–Jakob disease supports a pathogenic role for small PrPSc deposits common to the various molecular subtypes
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J. Hauw | J. Laplanche | S. Haïk | B. Faucheux | N. Privat | J. Brandel | V. Sazdovitch | V. Diouron | É. Morain | D. Salomon
[1] J. Ironside,et al. Correlation of Polydispersed Prion Protein and Characteristic Pathology in the Thalamus in Variant Creutzfeldt‐Jakob Disease: Implication of Small Oligomeric Species , 2011, Brain pathology.
[2] M. Bishop,et al. Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties , 2010, Proceedings of the National Academy of Sciences.
[3] W. Surewicz,et al. Molecular biology and pathology of prion strains in sporadic human prion diseases , 2010, Acta Neuropathologica.
[4] S. DeArmond,et al. PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases , 2010, Molecular Neurodegeneration.
[5] J. Langeveld,et al. Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics. , 2009, Brain : a journal of neurology.
[6] D. Harris,et al. Prion protein with an insertional mutation accumulates on axonal and dendritic plasmalemma and is associated with distinctive ultrastructural changes. , 2009, The American journal of pathology.
[7] B. Ghetti,et al. Incidence and spectrum of sporadic Creutzfeldt–Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification , 2009, Acta Neuropathologica.
[8] J. Hauw,et al. Loss of Cerebellar Granule Neurons Is Associated With Punctate but Not With Large Focal Deposits of Prion Protein in Creutzfeldt-Jakob Disease , 2009, Journal of neuropathology and experimental neurology.
[9] S. Lindquist,et al. Amyloid deposits: Protection against toxic protein species? , 2009, Cell cycle.
[10] M. Jeffrey,et al. Strain‐Associated Variations in Abnormal PrP Trafficking of Sheep Scrapie , 2008, Brain pathology.
[11] B. Ghetti,et al. Aggregated, Wild-Type Prion Protein Causes Neurological Dysfunction and Synaptic Abnormalities , 2008, The Journal of Neuroscience.
[12] S. Prusiner,et al. Cryo-Immunogold Electron Microscopy for Prions: Toward Identification of a Conversion Site , 2008, The Journal of Neuroscience.
[13] J. Hauw,et al. Regulating Factors of PrPres Glycosylation in Creutzfeldt-Jakob Disease - Implications for the Dissemination and the Diagnosis of Human Prion Strains , 2008, PloS one.
[14] Peter M. Douglas,et al. Chaperone-dependent amyloid assembly protects cells from prion toxicity , 2008, Proceedings of the National Academy of Sciences.
[15] T. Montine,et al. A novel human disease with abnormal prion protein sensitive to protease , 2008, Annals of neurology.
[16] J. Hauw,et al. Human prion diseases: from antibody screening to a standardized fast immunodiagnosis using automation , 2008, Modern Pathology.
[17] I. Ferrer. Synaptic pathology and cell death in the cerebellum in Creutzfeldt-Jakob disease , 2002, The Cerebellum.
[18] A. Hill,et al. Neurotoxic species in prion disease: a role for PrP isoforms? , 2007, Journal of neurochemistry.
[19] A. Aguzzi,et al. Insights into prion strains and neurotoxicity , 2007, Nature Reviews Molecular Cell Biology.
[20] B. Ghetti,et al. Accumulation of prion protein in the brain that is not associated with transmissible disease , 2007, Proceedings of the National Academy of Sciences.
[21] J Mackenzie,et al. Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease. , 2006, Brain : a journal of neurology.
[22] R. Castellani,et al. Classification of sporadic Creutzfeldt-Jakob disease revisited. , 2006, Brain : a journal of neurology.
[23] J. Ironside,et al. Phenotypic variability in human prion diseases , 2005, Neuropathology and applied neurobiology.
[24] H. Budka,et al. Subcellular localization of disease-associated prion protein in the human brain. , 2005, The American journal of pathology.
[25] S. DeArmond. Discovering the Mechanisms of Neurodegeneration in Prion Diseases , 2004, Neurochemical Research.
[26] B. Puig,et al. Clusterin solubility and aggregation in Creutzfeldt-Jakob disease , 2004, Acta Neuropathologica.
[27] E. Cochran,et al. Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders , 2004 .
[28] B. Ghetti,et al. Molecular Distinction between Pathogenic and Infectious Properties of the Prion Protein , 2003, Journal of Virology.
[29] T. Iwaki,et al. Increased clusterin (apolipoprotein J) expression in human and mouse brains infected with transmissible spongiform encephalopathies , 2002, Acta Neuropathologica.
[30] S. Prusiner,et al. Shattuck lecture--neurodegenerative diseases and prions. , 2001, The New England journal of medicine.
[31] B. Steinhoff,et al. Current clinical diagnosis in Creutzfeldt‐Jakob disease: Identification of uncommon variants , 2000, Annals of neurology.
[32] J. Hauw,et al. Neuropathologic variants of sporadic Creutzfeldt–Jakob disease and codon 129 of PrP gene , 2000, Neurology.
[33] A. Johnston,et al. Synapse loss associated with abnormal PrP precedes neuronal degeneration in the scrapie‐infected murine hippocampus , 2000, Neuropathology and applied neurobiology.
[34] P Brown,et al. Classification of sporadic Creutzfeldt‐Jakob disease based on molecular and phenotypic analysis of 300 subjects , 1999, Annals of neurology.
[35] Mark J. West,et al. Stereological methods for estimating the total number of neurons and synapses: issues of precision and bias , 1999, Trends in Neurosciences.
[36] J. Rossier,et al. Transmission of the BSE Agent to Mice in the Absence of Detectable Abnormal Prion Protein , 1997, Science.
[37] J Q Trojanowski,et al. Molecular basis of phenotypic variability in sporadc creudeldt‐jakob disease , 1996, Annals of neurology.
[38] J. Laplanche,et al. Molecular genetics of prion diseases in France , 1994, Neurology.
[39] M. Jeffrey,et al. Correlative light and electron microscopy studies of PrP localisation in 87V scrapie , 1994, Brain Research.
[40] J. Laplanche,et al. Molecular genetics of prion diseases in France. French Research Group on Epidemiology of Human Spongiform Encephalopathies. , 1994, Neurology.
[41] B. Brownell,et al. An ataxic form of subacute presenile polioencephalopathy (Creutzfeldt-Jakob disease) , 1965, Journal of neurology, neurosurgery, and psychiatry.