NR AVHJ
AU Isaacs,J.D.; Jackson,G.S.; Altmann,D.M.
TI The role of the cellular prion protein in the immune system
QU Clinical and Experimental Immunology 2006 Oct; 146(1): 1-8
PT journal article; research support, non-u.s. gov't; review
AB Prion protein (PrP) plays a key role in the pathogenesis of prion diseases. However, the normal function of the protein remains unclear. The cellular isoform (PrPc) is expressed widely in the immune system, in haematopoietic stem cells and mature lymphoid and myeloid compartments in addition to cells of the central nervous system. It is up-regulated in T cell activation and may be expressed at higher levels by specialized classes of lymphocyte. Furthermore, antibody cross-linking of surface PrP modulates T cell activation and leads to rearrangements of lipid raft constituents and increased phosphorylation of signalling proteins. These findings appear to indicate an important but, as yet, ill-defined role in T cell function. Although PrP(-/-) mice have been reported to have only minor alterations in immune function, recent work has suggested that PrP is required for self-renewal of haematopoietic stem cells. Here, we consider the evidence for a distinctive role for PrPc in the immune system and what the effects of anti-prion therapeutics may be on immune function.
ZR 105
MH Animals; Humans; Immunophenotyping; Lymphocyte Activation; Mice; PrPc Proteins/immunology/metabolism; Prions/*immunology/metabolism; T-Lymphocytes/*immunology/metabolism
AD Human Disease Immunogenetics Group, Department of Infectious Diseases and Immunity, Imperial College London, Hammersmith Hospital, London, UK
SP englisch
PO England