Prion protein: orchestrating neurotrophic activities.

摘要: PrP(C) is highly expressed in both the central and peripheral nervous systems from early stages of development adulthood. Its major conformational change conversion into an abnormal form (PrP(Sc)) has been associated with generation prions, infectious agent transmissible spongiform encephalopathies (TSEs). The massive neurodegeneration presented by individuals suffering these diseases gain neurotoxic activity PrP(Sc). On other hand, also observed transgenic mice expressing molecules deleted specific domains, which points to important functional domains within this molecule, supports hypothesis that loss-of function may contribute pathogenesis TSEs. Furthermore, a large body data demonstrates direct or indirect interaction extracellular matrix proteins, soluble factors, transmembrane G-protein coupled receptors ions channels. ability drive assembly multi-component complexes at cell surface likely basis for its neurotrophic functions. These properties indicate be relevant not only encephalopathies, but as ancillary component neurodegenerative diseases, therefore amenable therapeutic targeting.