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

B. A. Faucheux, E. Morain, V. Diouron, J.‐P. Brandel, D. Salomon, V. Sazdovitch, N. Privat, J.‐L. Laplanche, J.‐J. Hauw and S. Haïk (2011) Neuropathology and Applied Neurobiology37, 500–512

[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.