title: Role of cellular prion proteins in the function of macrophages and dendritic cells.
abstract: The cellular isoform of prion proteins (PrPC) is expressed in hematopoietic stem cells, granulocytes, T and B lymphocyte natural killer cells, platelets, monocytes, dendritic cells, and follicular dendritic cells, which may act as carrier cells for the spread of its abnormal isoform (PrPSc) before manifesting transmissible spongiform encephalopathies (TSEs). In particular, macrophages and dendritic cells seem to be involved in the replication of PrPSc after ingestion. In addition, information on the role of PrPC during phagocytotic activity in these cells has been obtained. A recent study showed that resident macrophages from ZrchI PrP gene (Prnp)-deficient (Prnp-/-) mice show augmented phagocytotic activity compared to Prnp+/+ counterparts. In contrast, our study suggests that Rikn Prnp-/- peritoneal macrophages show pseudopodium extension arrest and up-regulation of phagocytotic activity compared to Prnp+/+ cells. Although reports regarding phagocytotic activity in resident and peritoneal macrophages are inconsistent between ZrchI and Rikn Prnp-/- mice, it seems plausible that PrPC in macrophages could contribute to maintain the immunological environment. This review will introduce the recent progress in understanding the functions of PrPC in macrophages and dendritic cells under physiological conditions and its involvement in the pathogenesis of prion diseases.
authors.affiliation: Department of Molecular Immunology and Research Center for Food Safety, School of Agricultural and Life Sciences, Yayoi 1-1-1, Bunkyo-ku, Tokyo 113-8657, Japan.
authors.forename: Kayako
authors.initials: K
authors.lastname: Nitta
authors.forename: Akikazu
authors.initials: A
authors.lastname: Sakudo
authors.forename: Jun
authors.initials: J
authors.lastname: Masuyama
authors.forename: Guangai
authors.initials: G
authors.lastname: Xue
authors.forename: Katsuaki
authors.initials: K
authors.lastname: Sugiura
authors.forename: Takashi
authors.initials: T
authors.lastname: Onodera
mesh_terms: D000818:Animals; D003713:Dendritic Cells / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D005786:Gene Expression Regulation; D006801:Humans; D008264:Macrophages / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D010587:Phagocytosis; D018621:PrPC Proteins / Q000235:genetics* / Q000276:immunology*; D017096:Prion Diseases / Q000503:physiopathology*
chemical_list: D018621:PrPC Proteins