Prion protein heterogeneity in sporadic but not variant Creutzfeldt–Jakob disease: U.K. cases 1991–2002

• DOI: 10.1002/ana.20127
• Publication Date: 2004-05-27T21:02:33Z
• Authors: Mark W. Head; Tristan J. R. Bunn; Matthew T. Bishop; Victoria McLoughlin; Suzanne Lowrie; Clive S. McKimmie; Michelle C. Williams; Linda McCardle; Jan MacKenzie; Richard Knight; Robert G. Will; James W. Ironside
• Keywords: Creutzfeldt-Jakob Syndrome; Infectious agent; Kuru

Abstract Human prion diseases can occur as an idiopathic disorder (sporadic Creutzfeldt–Jakob disease) or be acquired, is the case for variant disease. These disorders are characterized by accumulation of a protease‐resistant form host‐encoded protein termed PrP Sc in brains affected individuals. has been proposed to principal, if not sole, component infectious agent, with its central nervous system primary event leading neurodegeneration. A major question remains whether self‐propagating structural differences might account clinicopathological diversity evident disease and different types underlie existence strains causative agent. Here, we describe results large‐scale biochemical study from autopsy‐proved cases (n = 59) compare these sporadic 170) United Kingdom over period 1991 2002. The show remarkably stereotyped. In contrast, considerable heterogeneity exists both between within Ann Neurol 2004;55:851–859