Prnp

Summary

Gene Symbol: Prnp
Description: prion protein
Alias: PrP, Prn, major prion protein, prion protein, PrP, prion protein, structural
Species: rat
Products:     Prnp

Top Publications

  1. Botto L, Masserini M, Cassetti A, Palestini P. Immunoseparation of Prion protein-enriched domains from other detergent-resistant membrane fractions, isolated from neuronal cells. FEBS Lett. 2004;557:143-7 pubmed
    ..Our results suggest that granule cells membranes contains different subtypes of detergent-resistant fractions, possibly deriving from different lipid rafts. ..
  2. Taylor D, Watt N, Perera W, Hooper N. Assigning functions to distinct regions of the N-terminus of the prion protein that are involved in its copper-stimulated, clathrin-dependent endocytosis. J Cell Sci. 2005;118:5141-53 pubmed
    The cellular prion protein (PrP(C)) is essential for the pathogenesis and transmission of prion diseases...
  3. Sarnataro D, Campana V, Paladino S, Stornaiuolo M, Nitsch L, Zurzolo C. PrP(C) association with lipid rafts in the early secretory pathway stabilizes its cellular conformation. Mol Biol Cell. 2004;15:4031-42 pubmed
    The pathological conversion of cellular prion protein (PrP(C)) into the scrapie prion protein (PrP(Sc)) isoform appears to have a central role in the pathogenesis of transmissible spongiform encephalopathies...
  4. Burns C, Aronoff Spencer E, Dunham C, Lario P, Avdievich N, Antholine W, et al. Molecular features of the copper binding sites in the octarepeat domain of the prion protein. Biochemistry. 2002;41:3991-4001 pubmed
    Recent evidence suggests that the prion protein (PrP) is a copper binding protein. The N-terminal region of human PrP contains four sequential copies of the highly conserved octarepeat sequence PHGGGWGQ spanning residues 60-91...
  5. Parkin E, Watt N, Hussain I, Eckman E, Eckman C, Manson J, et al. Cellular prion protein regulates beta-secretase cleavage of the Alzheimer's amyloid precursor protein. Proc Natl Acad Sci U S A. 2007;104:11062-7 pubmed
    ..The normal cellular function of the prion protein (PrP(C)), the causative agent of the transmissible spongiform encephalopathies such as Creutzfeldt-Jakob disease in ..
  6. Pantera B, Bini C, Cirri P, Paoli P, Camici G, Manao G, et al. PrPc activation induces neurite outgrowth and differentiation in PC12 cells: role for caveolin-1 in the signal transduction pathway. J Neurochem. 2009;110:194-207 pubmed publisher
    Cellular prion protein (PrP(c)) is a ubiquitous glycoprotein, whose physiological role is poorly characterized. It has been suggested that PrP(c) participates in neuritogenesis, neuroprotection, copper metabolism, and signal transduction...
  7. Wille H, Bian W, McDonald M, Kendall A, Colby D, Bloch L, et al. Natural and synthetic prion structure from X-ray fiber diffraction. Proc Natl Acad Sci U S A. 2009;106:16990-5 pubmed publisher
    A conformational isoform of the mammalian prion protein (PrP(Sc)) is the sole component of the infectious pathogen that causes the prion diseases...
  8. Galvan C, Camoletto P, Dotti C, Aguzzi A, Ledesma M. Proper axonal distribution of PrP(C) depends on cholesterol-sphingomyelin-enriched membrane domains and is developmentally regulated in hippocampal neurons. Mol Cell Neurosci. 2005;30:304-15 pubmed
    Defects in cellular localization and trafficking seem to facilitate the conversion of PrP(C) into the disease-associated form, PrP(Sc)...
  9. Lauren J, Gimbel D, Nygaard H, Gilbert J, Strittmatter S. Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers. Nature. 2009;457:1128-32 pubmed publisher
    ..Here we identify the cellular prion protein (PrP(C)) as an amyloid-beta-oligomer receptor by expression cloning...

More Information

Publications76

  1. Cobb N, SONNICHSEN F, McHaourab H, Surewicz W. Molecular architecture of human prion protein amyloid: a parallel, in-register beta-structure. Proc Natl Acad Sci U S A. 2007;104:18946-51 pubmed
    ..that are associated with conformational conversion of the normally monomeric and alpha-helical prion protein, PrP(C), to the beta-sheet-rich PrP(Sc)...
  2. Drisaldi B, Stewart R, Adles C, Stewart L, Quaglio E, Biasini E, et al. Mutant PrP is delayed in its exit from the endoplasmic reticulum, but neither wild-type nor mutant PrP undergoes retrotranslocation prior to proteasomal degradation. J Biol Chem. 2003;278:21732-43 pubmed
    ..address this issue, we have been using cultured cells to analyze the localization, biosynthesis, and metabolism of PrP molecules carrying mutations associated with familial prion diseases...
  3. Khan M, Sweeting B, Mulligan V, Arslan P, Cashman N, Pai E, et al. Prion disease susceptibility is affected by beta-structure folding propensity and local side-chain interactions in PrP. Proc Natl Acad Sci U S A. 2010;107:19808-13 pubmed publisher
    Prion diseases occur when the normally ?-helical prion protein (PrP) converts to a pathological ?-structured state with prion infectivity (PrP(Sc)). Exposure to PrP(Sc) from other mammals can catalyze this conversion...
  4. Um J, Nygaard H, Heiss J, Kostylev M, Stagi M, Vortmeyer A, et al. Alzheimer amyloid-? oligomer bound to postsynaptic prion protein activates Fyn to impair neurons. Nat Neurosci. 2012;15:1227-35 pubmed publisher
    Amyloid-beta (A?) oligomers are thought to trigger Alzheimer's disease pathophysiology. Cellular prion protein (PrP(C)) selectively binds oligomeric A? and can mediate Alzheimer's disease-related phenotypes...
  5. Barry A, Klyubin I, Mc Donald J, Mably A, Farrell M, Scott M, et al. Alzheimer's disease brain-derived amyloid-?-mediated inhibition of LTP in vivo is prevented by immunotargeting cellular prion protein. J Neurosci. 2011;31:7259-63 pubmed publisher
    Synthetic amyloid-? protein (A?) oligomers bind with high affinity to cellular prion protein (PrP(C)), but the role of this interaction in mediating the disruption of synaptic plasticity by such soluble A? in vitro is controversial...
  6. Gimbel D, Nygaard H, Coffey E, Gunther E, Lauren J, Gimbel Z, et al. Memory impairment in transgenic Alzheimer mice requires cellular prion protein. J Neurosci. 2010;30:6367-74 pubmed publisher
    ..Here, we crossed familial AD transgenes encoding APPswe and PSen1DeltaE9 into Prnp-/- mice to examine the necessity of PrP(C) for AD-related phenotypes...
  7. Pauly P, Harris D. Copper stimulates endocytosis of the prion protein. J Biol Chem. 1998;273:33107-10 pubmed
    ..This effect may be physiologically relevant and suggests the hypothesis that PrPC could serve as a recycling receptor for uptake of copper ions from the extracellular milieu. ..
  8. Farina F, Botto L, Chinello C, Cunati D, Magni F, Masserini M, et al. Characterization of prion protein-enriched domains, isolated from rat cerebellar granule cells in culture. J Neurochem. 2009;110:1038-48 pubmed publisher
    The biological functions of prion protein (PrP(C)) and its possible interaction with other specific molecular membrane partners remain largely unknown...
  9. Moleres F, Velayos J. The neurochemical nature of PrP(c)-containing cells in the rat brain. Brain Res. 2007;1174:143-51 pubmed
    The cellular prion protein (PrP(C)) is a membrane-bound glycoprotein abundantly expressed in neurons and glial cells within the CNS...
  10. Maloney B, Ge Y, Greig N, Lahiri D. Presence of a "CAGA box" in the APP gene unique to amyloid plaque-forming species and absent in all APLP-1/2 genes: implications in Alzheimer's disease. FASEB J. 2004;18:1288-90 pubmed publisher
  11. Urso E, Manno D, Serra A, Buccolieri A, Rizzello A, Danieli A, et al. Role of the cellular prion protein in the neuron adaptation strategy to copper deficiency. Cell Mol Neurobiol. 2012;32:989-1001 pubmed publisher
    Copper transporter 1 (CTR1), cellular prion protein (PrP(C)), natural resistance-associated macrophage protein 2 (NRAMP2) and ATP7A proteins control the cell absorption and efflux of copper (Cu) ions in nervous tissues upon physiological ..
  12. Bogdan A, Miyazawa M, Hashimoto K, Tsuji Y. Regulators of Iron Homeostasis: New Players in Metabolism, Cell Death, and Disease. Trends Biochem Sci. 2016;41:274-286 pubmed publisher
    ..We also discuss emerging roles of iron in apoptosis and a novel iron-dependent cell death pathway termed 'ferroptosis', the dysregulation of iron metabolism in human pathologies, and the use of iron chelators in cancer therapy. ..
  13. Walsh P, Simonetti K, Sharpe S. Core structure of amyloid fibrils formed by residues 106-126 of the human prion protein. Structure. 2009;17:417-26 pubmed publisher
    Peptides comprising residues 106-126 of the human prion protein (PrP) exhibit many features of the full-length protein...
  14. Antonyuk S, Trevitt C, Strange R, Jackson G, Sangar D, Batchelor M, et al. Crystal structure of human prion protein bound to a therapeutic antibody. Proc Natl Acad Sci U S A. 2009;106:2554-8 pubmed publisher
    Prion infection is characterized by the conversion of host cellular prion protein (PrP(C)) into disease-related conformers (PrP(Sc)) and can be arrested in vivo by passive immunization with anti-PrP monoclonal antibodies...
  15. Cabral A, Lee K, Martins V. Regulation of the cellular prion protein gene expression depends on chromatin conformation. J Biol Chem. 2002;277:5675-82 pubmed
    ..These new data indicate that PrPc gene regulation is highly dependent on disruption of chromatin fiber assembly, which allows some ubiquitous transcription factors accession to specific DNA elements. ..
  16. Azzalin A, Ferrara V, Arias A, Cerri S, Avella D, Pisu M, et al. Interaction between the cellular prion (PrPC) and the 2P domain K+ channel TREK-1 protein. Biochem Biophys Res Commun. 2006;346:108-15 pubmed
    The cellular prion protein (PrP(C)) is a highly conserved protein throughout the evolution of mammals and therefore is thought to play important cellular functions...
  17. Pham N, Sawyer T, Wang Y, Jazii F, Vair C, Taghibiglou C. Primary blast-induced traumatic brain injury in rats leads to increased prion protein in plasma: a potential biomarker for blast-induced traumatic brain injury. J Neurotrauma. 2015;32:58-65 pubmed publisher
    ..In conclusion, it appears that plasma levels of PrPC may be a novel biomarker for the detection of primary bTBI...
  18. Luo J, Wang S, Jian W, Chen C, Tang J, Lee C. Formation of amyloid fibrils from ?-amylase. FEBS Lett. 2012;586:680-5 pubmed publisher
    ..The features of ?-amylase proteins and fibrils are compared to prion proteins and fibrils. Furthermore, the cause of neurotoxicity in amyloid diseases is discussed. ..
  19. Li A, Harris D. Mammalian prion protein suppresses Bax-induced cell death in yeast. J Biol Chem. 2005;280:17430-4 pubmed
    Several lines of evidence suggest that PrP(C), the non-infectious form of the prion protein, may function to protect neurons and other cells from stress or toxicity...
  20. Lorenz H, Windl O, Kretzschmar H. Cellular phenotyping of secretory and nuclear prion proteins associated with inherited prion diseases. J Biol Chem. 2002;277:8508-16 pubmed
    The pathogenic mechanisms leading from mutations in the prion protein (PrP) gene to infectious disease are not understood...
  21. Nieznanski K, Nieznanska H, Skowronek K, Osiecka K, Stepkowski D. Direct interaction between prion protein and tubulin. Biochem Biophys Res Commun. 2005;334:403-11 pubmed
    Recently published data show that the prion protein in its cellular form (PrP(C)) is a component of multimolecular complexes...
  22. Um J, Kaufman A, Kostylev M, Heiss J, Stagi M, Takahashi H, et al. Metabotropic glutamate receptor 5 is a coreceptor for Alzheimer a? oligomer bound to cellular prion protein. Neuron. 2013;79:887-902 pubmed publisher
    ..A?o) trigger Alzheimer's disease (AD) pathophysiology and bind with high affinity to cellular prion protein (PrP(C))...
  23. Wu D, Zhang W, Luo Q, Luo K, Huang L, Wang W, et al. Copper (II) promotes the formation of soluble neurotoxic PrP oligomers in acidic environment. J Cell Biochem. 2010;111:627-33 pubmed publisher
    ..Recent studies identified soluble oligomers of PrP (prion protein) in damaged neuronal tissue. However, the details of PrP oligomerization are still unclear...
  24. Campana V, Caputo A, Sarnataro D, Paladino S, Tivodar S, Zurzolo C. Characterization of the properties and trafficking of an anchorless form of the prion protein. J Biol Chem. 2007;282:22747-56 pubmed
    Conversion of PrP(C) into PrP(Sc) is the central event in the pathogenesis of transmissible prion diseases...
  25. Keshet G, Ovadia H, Taraboulos A, Gabizon R. Scrapie-infected mice and PrP knockout mice share abnormal localization and activity of neuronal nitric oxide synthase. J Neurochem. 1999;72:1224-31 pubmed
    b>PrP(Sc), the only identified component of the scrapie prion, is a conformational isoform of PrPc. The physiological role of PrPc, a glycolipid-anchored glycoprotein, is still unknown...
  26. Gobbi M, Colombo L, Morbin M, Mazzoleni G, Accardo E, Vanoni M, et al. Gerstmann-Sträussler-Scheinker disease amyloid protein polymerizes according to the "dock-and-lock" model. J Biol Chem. 2006;281:843-9 pubmed
    Prion protein (PrP) amyloid formation is a central feature of genetic and acquired prion diseases such as Gerstmann-Sträussler-Scheinker disease (GSS) and variant Creutzfeldt-Jakob disease...
  27. Scalabrino G, Veber D, Mutti E, Calligaro A, Milani S, Tredici G. Cobalamin (vitamin B(12)) regulation of PrP(C), PrP(C)-mRNA and copper levels in rat central nervous system. Exp Neurol. 2012;233:380-90 pubmed publisher
    The pathogenesis of cobalamin (Cbl)-deficient (Cbl-D) neuropathy is not clear, nor is the role of prions (PrP(C)) in myelin maintenance...
  28. Strom A, Wang G, Reimer R, Finegood D, Scott F. Pronounced cytosolic aggregation of cellular prion protein in pancreatic beta-cells in response to hyperglycemia. Lab Invest. 2007;87:139-49 pubmed
    Cellular prion protein (PrP(C)), an N-linked glycoprotein, is expressed in a variety of tissues, but its functions remain unclear. PrP(C) is abundantly expressed in the endocrine pancreas, which regulates blood glucose homeostasis...
  29. Shyu W, Harn H, Saeki K, Kubosaki A, Matsumoto Y, Onodera T, et al. Molecular modulation of expression of prion protein by heat shock. Mol Neurobiol. 2002;26:1-12 pubmed
    ..with the conversion of the normal cellular form of the prion protein (PrPC) to an abnormal scrapie-isoform (PrP(Sc). The conversion of PrP(C) to PrP(Sc) is post-translational and is owing to protein conformational change...
  30. Rachidi W, Vilette D, Guiraud P, Arlotto M, Riondel J, Laude H, et al. Expression of prion protein increases cellular copper binding and antioxidant enzyme activities but not copper delivery. J Biol Chem. 2003;278:9064-72 pubmed
    The N-terminal region of the prion protein PrP(C) contains a series of octapeptide repeats. This region has been implicated in the binding of divalent metal ions, particularly copper...
  31. Zhang L, Li N, Wang Q, Fan B, Meng Q, Wu C. Cloning and sequencing of quail and pigeon prion genes. Anim Biotechnol. 2002;13:159-62 pubmed
    Quail and pigeon PrP genes were cloned and sequenced. Like mammalian PrP genes, quail and pigeon genes are encoded by a single exon of a single copy gene in the genome...
  32. Gaspersic J, Hafner Bratkovic I, Stephan M, Veranic P, Bencina M, Vorberg I, et al. Tetracysteine-tagged prion protein allows discrimination between the native and converted forms. FEBS J. 2010;277:2038-50 pubmed publisher
    The conformational conversion of prion protein (PrP) from a native conformation to the amyloid form is a hallmark of transmissible spongiform encephalopathies...
  33. Varela Nallar L, Toledo E, Larrondo L, Cabral A, Martins V, Inestrosa N. Induction of cellular prion protein gene expression by copper in neurons. Am J Physiol Cell Physiol. 2006;290:C271-81 pubmed
    ..In addition, copper induced the activity of a reporter vector driven by the rat PrPC gene (Prnp) promoter stably transfected into PC12 cells, whereas no effect was observed in glial C6 clones...
  34. Walsh P, Vanderlee G, Yau J, Campeau J, Sim V, Yip C, et al. The mechanism of membrane disruption by cytotoxic amyloid oligomers formed by prion protein(106-126) is dependent on bilayer composition. J Biol Chem. 2014;289:10419-30 pubmed publisher
    ..Here, we describe the physical basis for the cytotoxicity of oligomers formed by the prion protein (PrP)-derived amyloid peptide PrP(106-126)...
  35. Smirnovas V, Baron G, Offerdahl D, Raymond G, Caughey B, Surewicz W. Structural organization of brain-derived mammalian prions examined by hydrogen-deuterium exchange. Nat Struct Mol Biol. 2011;18:504-6 pubmed publisher
    One of the mysteries in prion research is the structure of the infectious form of mammalian prion protein PrP(Sc). Here we used mass spectrometry analysis of hydrogen-deuterium exchange to examine brain-derived PrP(Sc)...
  36. Loberto N, Prioni S, Bettiga A, Chigorno V, Prinetti A, Sonnino S. The membrane environment of endogenous cellular prion protein in primary rat cerebellar neurons. J Neurochem. 2005;95:771-83 pubmed
    ..Thus, prion protein plasma membrane environment in differentiated neurons resulted to be a complex entity, whose integrity requires a network of lipid-mediated non-covalent interactions...
  37. Hu W, Nessler S, Hemmer B, Eagar T, Kane L, Leliveld S, et al. Pharmacological prion protein silencing accelerates central nervous system autoimmune disease via T cell receptor signalling. Brain. 2010;133:375-88 pubmed publisher
    ..Our data identify cellular prion protein as a regulator of cellular immunological homoeostasis and suggest cellular prion protein as a novel potential target for therapeutic immunomodulation...
  38. Monnet C, Marthiens V, Enslen H, Frobert Y, Sobel A, Mège R. Heterogeneity and regulation of cellular prion protein glycoforms in neuronal cell lines. Eur J Neurosci. 2003;18:542-8 pubmed
  39. Heiseke A, Schöbel S, Lichtenthaler S, Vorberg I, Groschup M, Kretzschmar H, et al. The novel sorting nexin SNX33 interferes with cellular PrP formation by modulation of PrP shedding. Traffic. 2008;9:1116-29 pubmed publisher
    The cellular prion protein (PrP(c)) is a glycosyl-phosphatidylinositol (GPI)-anchored protein trafficking in the secretory and endocytic pathway and localized mainly at the plasma membrane...
  40. Goldfarb L, Brown P, Mitrova E, Cervenakova L, Goldin L, Korczyn A, et al. Creutzfeldt-Jacob disease associated with the PRNP codon 200Lys mutation: an analysis of 45 families. Eur J Epidemiol. 1991;7:477-86 pubmed
    200Lys mutation in the human PRNP coding region has been identified in 45 of the 55 CJD-affected families thus far presented to our NIH laboratory...
  41. Beck K, Kay J, Braun J. Rdj2, a J protein family member, interacts with cellular prion PrP(C). Biochem Biophys Res Commun. 2006;346:866-71 pubmed
    b>PrP(C) is a glycosylphosphatidylinositol (GPI) anchored glycoprotein of unknown function. Misfolding of normal cellular PrP(C) to the pathogenic PrP(Sc) is the hallmark of prion diseases (transmissible spongiform encephalopathies)...
  42. Liao T, Lee L, Chen R. Leu138 in bovine prion peptide fibrils is involved in seeding discrimination related to codon 129 M/V polymorphism in the prion peptide seeding experiment. FEBS J. 2011;278:4351-61 pubmed publisher
    ..Here, we used a short prion protein (PrP) peptide as a model system to test whether seeding discrimination can be found in this simple system...
  43. Botto L, Cunati D, Coco S, Sesana S, Bulbarelli A, Biasini E, et al. Role of lipid rafts and GM1 in the segregation and processing of prion protein. PLoS ONE. 2014;9:e98344 pubmed publisher
    ..Our data provide new insights into the cellular mechanism of prion conversion and suggest that GM1-prion protein interaction at the cell surface could play a significant role in the mechanism predisposing to pathology. ..
  44. Rouvinski A, Gahali Sass I, Stav I, Metzer E, Atlan H, Taraboulos A. Both raft- and non-raft proteins associate with CHAPS-insoluble complexes: some APP in large complexes. Biochem Biophys Res Commun. 2003;308:750-8 pubmed
    ..Large complexes were enriched in caveolin and ESA. Medium size complexes were enriched in PrP, whereas small complexes contained non-raft proteins, PrP, and some ESA...
  45. Coitinho A, Freitas A, Lopes M, Hajj G, Roesler R, Walz R, et al. The interaction between prion protein and laminin modulates memory consolidation. Eur J Neurosci. 2006;24:3255-64 pubmed
    ..Our findings show that, through its interaction with LN, hippocampal PrPc plays a critical role in memory processing and suggest that this role is mediated by activation of both PKA and ERK1/2 signaling pathways. ..
  46. Rushworth J, Griffiths H, Watt N, Hooper N. Prion protein-mediated toxicity of amyloid-? oligomers requires lipid rafts and the transmembrane LRP1. J Biol Chem. 2013;288:8935-51 pubmed publisher
    ..The cellular prion protein (PrP(C)) was recently identified as a high affinity neuronal receptor for A? oligomers...
  47. Chiarini L, Freitas A, Zanata S, Brentani R, Martins V, Linden R. Cellular prion protein transduces neuroprotective signals. EMBO J. 2002;21:3317-26 pubmed
    To test for a role for the cellular prion protein (PrP(c)) in cell death, we used a PrP(c)-binding peptide. Retinal explants from neonatal rats or mice were kept in vitro for 24 h, and anisomycin (ANI) was used to induce apoptosis...
  48. Wopfner F, Weidenhöfer G, Schneider R, von Brunn A, Gilch S, Schwarz T, et al. Analysis of 27 mammalian and 9 avian PrPs reveals high conservation of flexible regions of the prion protein. J Mol Biol. 1999;289:1163-78 pubmed
    ..The function of PrPcand the tertiary structure of PrPScare unclear. Various data indicate which parts of PrP might control the species barrier in prion diseases and the binding of putative factors to PrP...
  49. Marcos Z, Bodegas M, Sesma M, Guembe L. Comparative study of PrPc expression in rat, monkey, and cow gastrointestinal tract. Ann N Y Acad Sci. 2005;1040:391-4 pubmed
    The gastrointestinal tract (GIT) appears to be the main site of entry for the pathological isoform of prions (PrP(sc))...
  50. Saeki K, Hirota Y, Matsumoto Y, Onodera T. Three-exon structure of the gene encoding the rat prion protein and its expression in tissues. Virus Genes. 1996;12:15-20 pubmed
    The prion protein (PrP), encoded by a chromosomal gene, is associated with development of the neurodegeneration of prion-induced diseases...
  51. Inayathullah M, Satheeshkumar K, Malkovskiy A, Carre A, Sivanesan S, Hardesty J, et al. Solvent microenvironments and copper binding alters the conformation and toxicity of a prion fragment. PLoS ONE. 2013;8:e85160 pubmed publisher
    ..The secondary structure of a prion fragment, PrP(111-126), was determined using circular dichroism (CD) spectroscopy in various microenvironments...
  52. Pham N, Dhar A, Khalaj S, Desai K, Taghibiglou C. Down regulation of brain cellular prion protein in an animal model of insulin resistance: possible implication in increased prevalence of stroke in pre-diabetics/diabetics. Biochem Biophys Res Commun. 2014;448:151-6 pubmed publisher
    ..Since one of the physiological functions of the cellular prion protein (PrP(C)) is neuroprotection, we studied effects of brain insulin resistance on the expression of PrP(C) in fructose-fed ..
  53. Guillot Sestier M, Sunyach C, Druon C, Scarzello S, Checler F. The alpha-secretase-derived N-terminal product of cellular prion, N1, displays neuroprotective function in vitro and in vivo. J Biol Chem. 2009;284:35973-86 pubmed publisher
    Cellular prion protein (PrP(c)) undergoes a disintegrin-mediated physiological cleavage, generating a soluble amino-terminal fragment (N1), the function of which remained unknown...
  54. Stevens D, Walter E, Rodr guez A, Draper D, Davies P, Brown D, et al. Early onset prion disease from octarepeat expansion correlates with copper binding properties. PLoS Pathog. 2009;5:e1000390 pubmed publisher
    Insertional mutations leading to expansion of the octarepeat domain of the prion protein (PrP) are directly linked to prion disease...
  55. Melo J, Agostinho P, Oliveira C. Prion protein aggregation and neurotoxicity in cortical neurons. Ann N Y Acad Sci. 2007;1096:220-9 pubmed
    ..A cellular glycoprotein, PrP(C) is converted in an altered isoform, PrP(Sc), that accumulates in the brain, and is believed to be responsible ..
  56. Sarnataro D, Caputo A, Casanova P, Puri C, Paladino S, Tivodar S, et al. Lipid rafts and clathrin cooperate in the internalization of PrP in epithelial FRT cells. PLoS ONE. 2009;4:e5829 pubmed publisher
    The cellular prion protein (PrP(C)) plays a key role in the pathogenesis of Transmissible Spongiform Encephalopathies in which the protein undergoes post-translational conversion to the infectious form (PrP(Sc))...
  57. Zajkowski T, Nieznanska H, Nieznanski K. Stabilization of microtubular cytoskeleton protects neurons from toxicity of N-terminal fragment of cytosolic prion protein. Biochim Biophys Acta. 2015;1853:2228-39 pubmed publisher
    Prion protein (PrP) mislocalized in the cytosol has been presumed to be the toxic entity responsible for the neurodegenerative process in transmissible spongiform encephalopathies (TSE), also called prion diseases...
  58. Carneiro M, Americo T, Guimarães M, Linden R. The prion protein selectively binds to and modulates the content of purinergic receptor P2X4R. Biochem Biophys Res Commun. 2016;472:293-8 pubmed publisher
    The GPI-anchored prion protein (PrP(C)) is involved in neurodegeneration, either through misfolding in the Transmissible Spongiform Encephalopathies (TSE), or as a mediator of the neurotoxicity of peptide oligomers in Alzheimer's Disease...
  59. Scalabrino G, Mutti E, Veber D, Rodriguez Menendez V, Novembrino C, Calligaro A, et al. The octapeptide repeat PrP(C) region and cobalamin-deficient polyneuropathy of the rat. Muscle Nerve. 2011;44:957-67 pubmed publisher
    ..We investigated whether the octapeptide repeat (OR) region of prion protein (PrP(C)) (which is claimed to have myelinotrophic properties) is involved in the pathogenesis of rat Cbl-deficient (Cbl-..
  60. Saeki K, Matsumoto Y, Onodera T. Identification of a promoter region in the rat prion protein gene. Biochem Biophys Res Commun. 1996;219:47-52 pubmed
    ..Deletion analysis showed that an inverted CCAAt and adjoining Sp-1 binding sequences may play an important role in transcription of the RaPrP gene. ..
  61. Mattei V, Martellucci S, Santilli F, Manganelli V, Garofalo T, Candelise N, et al. Morphine Withdrawal Modifies Prion Protein Expression in Rat Hippocampus. PLoS ONE. 2017;12:e0169571 pubmed publisher
    ..We speculate that this might be the mechanism by which stressful stimuli induced by opiate withdrawal and the subsequent long-term homeostatic changes in hippocampal plasticity, modulate the expression and the dynamics of Prion protein. ..
  62. Mercer R, Ma L, Watts J, Strome R, Wohlgemuth S, Yang J, et al. The prion protein modulates A-type K+ currents mediated by Kv4.2 complexes through dipeptidyl aminopeptidase-like protein 6. J Biol Chem. 2013;288:37241-55 pubmed publisher
    Widely expressed in the adult central nervous system, the cellular prion protein (PrP(C)) is implicated in a variety of processes, including neuronal excitability...
  63. Salmona M, Morbin M, Massignan T, Colombo L, Mazzoleni G, Capobianco R, et al. Structural properties of Gerstmann-Straussler-Scheinker disease amyloid protein. J Biol Chem. 2003;278:48146-53 pubmed
    Prion protein (PrP) amyloid formation is a central feature of genetic and acquired forms of prion disease such as Gerstmann-Sträussler-Scheinker disease (GSS) and variant Creutzfeldt-Jakob disease...
  64. Vanderperre B, Staskevicius A, Tremblay G, McCoy M, O Neill M, Cashman N, et al. An overlapping reading frame in the PRNP gene encodes a novel polypeptide distinct from the prion protein. FASEB J. 2011;25:2373-86 pubmed publisher
    The prion protein gene PRNP directs the synthesis of one of the most intensively studied mammalian proteins, the prion protein (PrP)...
  65. Kazlauskaite J, Young A, Gardner C, Macpherson J, Venien Bryan C, Pinheiro T. An unusual soluble beta-turn-rich conformation of prion is involved in fibril formation and toxic to neuronal cells. Biochem Biophys Res Commun. 2005;328:292-305 pubmed
    A key molecular event in prion diseases is the conversion of the prion protein (PrP) from its normal cellular form (PrPC) to the disease-specific form (PrPSc)...
  66. Gomi H, Ikeda T, Kunieda T, Itohara S, Prusiner S, Yamanouchi K. Prion protein (PrP) is not involved in the pathogenesis of spongiform encephalopathy in zitter rats. Neurosci Lett. 1994;166:171-4 pubmed
    In order to elucidate the relationship between the prion protein (PrP) structure and the development of spongiform encephalopathy in zitter rats, we analyzed the nucleotide sequences and restriction fragment length variation (RFLV) of ..
  67. Brugger B, Graham C, Leibrecht I, Mombelli E, Jen A, Wieland F, et al. The membrane domains occupied by glycosylphosphatidylinositol-anchored prion protein and Thy-1 differ in lipid composition. J Biol Chem. 2004;279:7530-6 pubmed
    ..These results further identify unsaturation and glycosylation of lipids as major sources of diversity of raft structure. ..