Abstract
Prion diseases are transmissible and fatal neurodegenerative disorders which involve infiltration and activation of mononuclear phagocytes at the brain lesions. A 20-aa acid fragment of the human cellular prion protein, PrP(106-126), was reported to mimic the biological activity of the pathologic isoform of prion and activates mononuclear phagocytes. The cell surface receptor(s) mediating the activity of PrP(106-126) is unknown. In this study, we show that PrP(106-126) is chemotactic for human monocytes through the use of a G protein-coupled receptor formyl peptide receptor-like 1 (FPRL1), which has been reported to interact with a diverse array of exogenous or endogenous ligands. Upon stimulation by PrP(106-126), FPRL1 underwent a rapid internalization and, furthermore, PrP(106-126) enhanced monocyte production of proinflammatory cytokines, which was inhibited by pertussis toxin. Thus, FPRL1 may act as a "pattern recognition" receptor that interacts with multiple pathologic agents and may be involved in the proinflammatory process of prion diseases.
Publication types
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Animals
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Calcium Signaling / immunology
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Cell Line
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Chemotactic Factors / physiology*
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Chemotactic Factors / toxicity
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Chemotaxis, Leukocyte / immunology*
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Cytokines / biosynthesis
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GTP-Binding Proteins / metabolism*
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Humans
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Inflammation / immunology
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Inflammation / metabolism
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Ligands
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Microglia / immunology
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Microglia / metabolism
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Monocytes / immunology
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Monocytes / metabolism
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N-Formylmethionine Leucyl-Phenylalanine / metabolism*
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Peptide Fragments / agonists*
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Peptide Fragments / physiology
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Peptide Fragments / toxicity*
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Prions / agonists*
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Prions / physiology
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Prions / toxicity*
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Rats
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Receptors, Formyl Peptide
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Receptors, Immunologic / metabolism
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Receptors, Immunologic / physiology*
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Receptors, Lipoxin*
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Receptors, Peptide / metabolism
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Receptors, Peptide / physiology*
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Tumor Cells, Cultured
Substances
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Chemotactic Factors
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Cytokines
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FPR2 protein, human
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Ligands
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Peptide Fragments
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Prions
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Receptors, Formyl Peptide
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Receptors, Immunologic
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Receptors, Lipoxin
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Receptors, Peptide
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prion protein (106-126)
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N-Formylmethionine Leucyl-Phenylalanine
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GTP-Binding Proteins