Spongiform encephalopathies: insights from transgenic models.

摘要: Publisher Summary This chapter summarizes some of the transgenic mouse models that contributed to current understanding pathogenesis transmissible spongiform encephalopathies. Prion diseases, or encephalopathies, are neurological disorders caused by pathogens termed prions. Human prion diseases characterized extended incubation periods ranging from several months decades followed a progressive clinical phase presenting with severe dementia and ataxia. Clinical disease is always lethal: death can occur within as short period few weeks but occasionally in up years. Peripheral pathogenesis, ultimately neuroinvasion dependent on components host-immune system. Collectively, these processes require either B cells their products. At least one cell-dependent event acquisition functional follicular dendritic cell network germinal centers peripheral lymphoid tissue. These major sites extraneuronal protein (PrP c ) expression probably principal PrP Sc accumulation.