The complement system in prion diseases.
The complement system is an essential component of the innate immune system that aids the recognition and destruction of pathogens. Despite the potent cytolytic activity of this system, some pathogens have turned interactions with complement to their advantage. Transmissible spongiform encephalopathies (TSEs), or prion diseases, also seem to use the complement system to facilitate target cell infection in lymphoid tissues. TSEs are associated with the accumulation of disease-specific prion protein in the brain, which is accompanied by progressive neurodegeneration. Unregulated activation of complement can cause host tissue damage, as observed in some neurodegenerative disorders, and active complement components have been detected in the brains of hosts with TSEs. Whether complement inhibitors will be useful to treat TSEs remains to be determined.
Mabbott
Neil A
NA
Institute for Animal Health, Neuropathogenesis Unit, Ogston Building, West Mains Road, Edinburgh EH9 3JF, UK. neil.mabbott@bbsrc.ac.uk
D000818:Animals; D002490:Central Nervous System / Q000276:immunology / Q000503:physiopathology; D003167:Complement Activation / Q000502:physiology; D003165:Complement System Proteins / Q000502:physiology*; D006801:Humans; D018621:PrPC Proteins / Q000502:physiology; D017096:Prion Diseases / Q000503:physiopathology*; D015398:Signal Transduction / Q000502:physiology*
D018621:PrPC Proteins; D003165:Complement System Proteins