Multiple prion types in the same brain Is a molecular diagnosis of CJD possible

摘要: Transmissible spongiform encephalopathies (TSEs) are rare neurologic disorders that bear some resemblance to more common neurodegenerative conditions, such as Alzheimer’s disease and Parkinson’s disease. TSEs, however, transmissible, whereas there is no convincing evidence AD or PD caused by transmissible agents.1 Conversely, AD, all associated with progressive accumulation of fibrillar proteins in the brain. In TSE, most suggests prion protein (PrP) cause agent.2 two types involved; extracellular amyloid deposits composed Aβ, a proteolytic fragment precursor protein, intracellular neurofibrillary inclusions τ microtubule protein. PD, aggregates synuclein form Lewy bodies, histologic hallmark PD. This tendency normal cellular acquire novel attributes, which may be related conformational changes favor formation fibrillar, amyloid-like aggregates, mechanism pathogenesis these disorders.3 The TSEs often sporadic disorders, but hereditary iatrogenic variants also recognized.1 The TSE Creutzfeldt-Jakob (CJD). Hereditary forms include Gerstmann-Straussler-Sheinker syndrome (GSS), familial CJD, fatal insomnia (FFI). resulted from administration growth hormone derived cadaver pituitaries, practice longer used. Of great public health concern epidemic bovine encephalopathy (BSE), appears etiologically linked new …