Microglia and the pathogenesis of spongiform encephalopathies

摘要: Alterations in the phenotype and function of microglia, resident mononuclear phagocytes central nervous system, are among earliest indications pathology within brain spinal cord. The prion diseases, also known as spongiform encephalopathies, fatal neurodegenerative disorders with sporadic, genetic or acquired infectious manifestations. A hallmark all diseases is aberrant metabolism resulting accumulation protein. Conversion normal cellular protein [PrP(c)] into abnormal pathogenic (or disease-causing) isoform [PrP(Sc)] involves a conformational alteration whereby alpha-helical content transformed beta-sheet. histological characteristics these change, astrocytosis, neuronal loss progressive protease-resistant isoform. An additional upregulation microglial response has been reported Kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome (GSS), scrapie, transgenic murine models culture, where activation often accompanies deposition loss. This article will review roles microglia encephalopathies.