NR AYEI
AU Wadsworth,J.D.F.; Collinge,J.
TI Update on human prion disease
QU Biochimica et Biophysica Acta - Molecular Basis of Disease 2007 Jun; 1772(6): 598-609
PT journal article; review
AB The recognition that variant Creutzfeldt-Jakob disease (vCJD) is caused by the same prion strain as bovine spongiform encephalopathy in cattle has dramatically highlighted the need for a precise understanding of the molecular biology of human prion diseases. Detailed clinical, pathological and molecular data from a large number of human prion disease patients indicate that phenotypic diversity in human prion disease relates in part to the propagation of disease-related PrP isoforms with distinct physicochemical properties. Incubation periods of prion infection in humans can exceed 50 years and therefore it will be some years before the extent of any human vCJD epidemic can be predicted with confidence.
ZR 155
MH Animals; Brain/metabolism/pathology; Cattle; Creutzfeldt-Jakob Syndrome/metabolism/pathology; Encephalopathy, Bovine Spongiform/metabolism/pathology; Humans; Models, Biological; Prion Diseases/*metabolism/pathology; Prions/*metabolism
AD MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College London, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.
SP englisch
PO Niederlande