PRNP

Summary

Gene Symbol: PRNP
Description: prion protein
Alias: PRPC, Prp, SIP, major prion protein, prion protein (p27-30) (Creutzfeldt-Jakob disease, Gerstmann-Strausler-Scheinker syndrome, fatal familial insomnia)
Species: sheep

Publications

  1. Different scrapie-associated fibril proteins (PrP) are encoded by lines of sheep selected for different alleles of the Sip gene
    W Goldmann
    Institute for Animal Health, AFRC and MRC Neuropathogenesis Unit, Edinburgh
    J Gen Virol 72:2411-7
  2. Sheep scrapie susceptibility-linked polymorphisms do not modulate the initial binding of cellular to disease-associated prion protein prior to conversion
    Alan Rigter
    Central Institute for Animal Disease Control, Department of Bacteriology and TSEs, PO Box 2004, 8203 AA Lelystad, The Netherlands
    J Gen Virol 86:2627-34
  3. Structural differences between allelic variants of the ovine prion protein revealed by molecular dynamics simulations
    Raymond Bujdoso
    Centre for Veterinary Science, Department of Veterinary Medicine, University of Cambridge, Cambridge, CB3 OES, UK
    Proteins 61:840-9
  4. Identification of a new leucine haplotype (ALQ) at codon 154 in the ovine prion protein gene in Spanish sheep
    L Alvarez
    Departamento de Producción Animal I, Universidad de León, 24071 León, Spain
    J Anim Sci 84:259-65
  5. Prion protein gene (PRNP) polymorphisms in Xinjiang local sheep breeds in China
    Z Lan
    Life Sciences and Technology College, Ocean University of China, Qingdao, P R China
    Arch Virol 151:2095-101
  6. Characterization of proteinase K-resistant N- and C-terminally truncated PrP in Nor98 atypical scrapie
    Mikael Klingeborn
    Department of Molecular Biosciences, Faculty of Veterinary Medicine and Animal Science, Swedish University of Agricultural Sciences, Biomedical Centre, Box 588, S 751 23 Uppsala, Sweden
    J Gen Virol 87:1751-60
  7. Detection of prion protein gene polymorphisms in Baltic breeds of sheep
    E Sild
    Institute of Animal Science, Estonian Agricultural University, Kreutzwaldi 1, 51014 Tartu, Estonia
    Vet Rec 159:247-50
  8. Associations of polymorphisms of the ovine prion protein gene with growth, carcass, and computerized tomography traits in Scottish Blackface lambs
    R M Sawalha
    Scottish Agricultural College, West Mains Road, Edinburgh EH9 3JG, United Kingdom
    J Anim Sci 85:632-40
  9. Linkage disequilibrium across six prion gene regions spanning 20 kbp in U.S. sheep
    Benedict T Green
    U S Department of Agriculture, Agricultural Research Service, U S Meat Animal Research Center, State Spur 18D, P O Box 166, Clay Center, Nebraska 68933, USA
    Mamm Genome 17:1121-9
  10. Ovine prion protein variant A(136)R(154)L(168)Q(171) increases resistance to experimental challenge with bovine spongiform encephalopathy agent
    Wilfred Goldmann
    Institute for Animal Health IAH, Neuropathogenesis Unit, West Mains Road, Edinburgh EH9 3JF, UK
    J Gen Virol 87:3741-5

Scientific Experts

  • W Goldmann
  • J A Kornblatt
  • Charalambos Billinis
  • Raymond Bujdoso
  • Sujeong Yang
  • Tim J Fitzmaurice
  • M E Babar
  • Martin Jeffrey
  • Evelyne Lampo
  • Sarah Jo Moore
  • Alana M Thackray
  • Yiqin Wang
  • Isabelle Palhiere
  • R M Sawalha
  • Patama Thumdee
  • L Alvarez
  • E Sild
  • Benedict T Green
  • Z Lan
  • Mikael Klingeborn
  • Dominikus A Lysek
  • Heath Ecroyd
  • Alan Rigter
  • Bryan Maloney
  • L F Haire
  • C M Seabury
  • M Maissen
  • G Vaccari
  • T V Baszler
  • Luc J Peelman
  • A Nadeem
  • Tim Erkens
  • M Imran
  • J Ahmad
  • B F Benkel
  • Jo Vandesompele
  • Gillian McGovern
  • Mario Van Poucke
  • A Farid
  • Caroline M Goodsir
  • D Westaway
  • Alex Van Zeveren
  • Yves Amigues
  • Cyril Leymarie
  • Katayoun Moazami Goudarzi
  • Zhenkui Qin
  • Thais Pantano
  • Marion Simmons
  • Melanie Chaplin
  • John Spiropoulos
  • David F Burke
  • Lee Hopkins
  • Etienne Neuts
  • Etienne Verrier
  • Edmond Paul Cribiu
  • Man-Sun Sy
  • Junwen Qiao
  • Man Sun Sy
  • Shuiliang Yu
  • Deming Zhao
  • Korakot Nganvongpanit
  • Bernhard Gehrig
  • G Simm
  • S Brotherstone
  • Siriluck Ponsuksili
  • Michael Hoelker
  • Dawit Tesfaye
  • W Y N Man
  • Markus Gilles
  • Eduard Murani
  • Danyel Jennen
  • Karl Schellander
  • J Conington
  • Josef Griese
  • B Villanueva
  • Klaus Wimmers
  • J J Arranz
  • William W Laegreid
  • I Y Lee
  • Z Grislis
  • X Zhang
  • I Miceikiene
  • F San Primitivo
  • H Viinalass
  • Z L Wang
  • Lotta Wik
  • Y Liu
  • P B Belt
  • Michael P Heaton
  • R Volskiene

Detail Information

Publications38

  1. Different scrapie-associated fibril proteins (PrP) are encoded by lines of sheep selected for different alleles of the Sip gene
    W Goldmann
    Institute for Animal Health, AFRC and MRC Neuropathogenesis Unit, Edinburgh
    J Gen Virol 72:2411-7
    ..Restriction fragment length polymorphisms of the scrapie-associated fibril protein (PrP) gene are associated with the Sip alleles...
  2. Sheep scrapie susceptibility-linked polymorphisms do not modulate the initial binding of cellular to disease-associated prion protein prior to conversion
    Alan Rigter
    Central Institute for Animal Disease Control, Department of Bacteriology and TSEs, PO Box 2004, 8203 AA Lelystad, The Netherlands
    J Gen Virol 86:2627-34
    Conversion of the host-encoded protease-sensitive cellular prion protein (PrPC) into the scrapie-associated protease-resistant isoform (PrPSc) of prion protein (PrP) is the central event in transmissible spongiform encephalopathies or ..
  3. Structural differences between allelic variants of the ovine prion protein revealed by molecular dynamics simulations
    Raymond Bujdoso
    Centre for Veterinary Science, Department of Veterinary Medicine, University of Cambridge, Cambridge, CB3 OES, UK
    Proteins 61:840-9
    We have modeled ovine prion protein (residues 119-233) based on NMR structures of PrP from other mammalian species...
  4. Identification of a new leucine haplotype (ALQ) at codon 154 in the ovine prion protein gene in Spanish sheep
    L Alvarez
    Departamento de Producción Animal I, Universidad de León, 24071 León, Spain
    J Anim Sci 84:259-65
    Genetic susceptibility to scrapie is closely linked to variations at codons 136, 154, and 171 of the prion protein (PRNP) gene...
  5. Prion protein gene (PRNP) polymorphisms in Xinjiang local sheep breeds in China
    Z Lan
    Life Sciences and Technology College, Ocean University of China, Qingdao, P R China
    Arch Virol 151:2095-101
    Amino acid polymorphisms of the prion protein (PrP) gene (PRNP), particularly those occurring at codons 136, 154, and 171 have a significant influence on scrapie pathogenesis in many sheep breeds...
  6. Characterization of proteinase K-resistant N- and C-terminally truncated PrP in Nor98 atypical scrapie
    Mikael Klingeborn
    Department of Molecular Biosciences, Faculty of Veterinary Medicine and Animal Science, Swedish University of Agricultural Sciences, Biomedical Centre, Box 588, S 751 23 Uppsala, Sweden
    J Gen Virol 87:1751-60
    ..Here, the proteinase K (PK)-resistant prion protein (PrP) fragments from two Swedish cases of Nor98 atypical scrapie have been characterized...
  7. Detection of prion protein gene polymorphisms in Baltic breeds of sheep
    E Sild
    Institute of Animal Science, Estonian Agricultural University, Kreutzwaldi 1, 51014 Tartu, Estonia
    Vet Rec 159:247-50
    ..sheep kept isolated on the Estonian island of Ruhnu, were sequence-analysed for polymorphisms in the prion protein (PrP) gene, to determine their genotype and the allele frequencies of polymorphisms in PrP known to confer resistance to ..
  8. Associations of polymorphisms of the ovine prion protein gene with growth, carcass, and computerized tomography traits in Scottish Blackface lambs
    R M Sawalha
    Scottish Agricultural College, West Mains Road, Edinburgh EH9 3JG, United Kingdom
    J Anim Sci 85:632-40
    The objective of this study was to investigate and estimate the associations of the ovine prion protein (PrP) genotypes with a wide range of performance traits in Scottish Blackface lambs...
  9. Linkage disequilibrium across six prion gene regions spanning 20 kbp in U.S. sheep
    Benedict T Green
    U S Department of Agriculture, Agricultural Research Service, U S Meat Animal Research Center, State Spur 18D, P O Box 166, Clay Center, Nebraska 68933, USA
    Mamm Genome 17:1121-9
    Single nucleotide polymorphisms (SNPs) and haplotype alleles within the prion gene (PRNP) coding sequence of domestic sheep (Ovis aries) are associated with genetic predisposition to scrapie, a transmissible spongiform encephalopathy ..
  10. Ovine prion protein variant A(136)R(154)L(168)Q(171) increases resistance to experimental challenge with bovine spongiform encephalopathy agent
    Wilfred Goldmann
    Institute for Animal Health IAH, Neuropathogenesis Unit, West Mains Road, Edinburgh EH9 3JF, UK
    J Gen Virol 87:3741-5
    ..and incubation periods of transmissible spongiform encephalopathies, such as scrapie in sheep, are modulated by the PrP gene...
  11. Expression of the prion protein gene (PRNP) and cellular prion protein (PrPc) in cattle and sheep fetuses and maternal tissues during pregnancy
    Patama Thumdee
    Institute of Animal Science, Animal Breeding and Husbandry Group, University of Bonn, Bonn, Germany
    Gene Expr 13:283-97
    ..In cattle, prion protein gene (PRNP) transcripts were detected by semiquantitative RT-PCR in reproductive tissues such as ovary, oviduct, endometrium, ..
  12. The stability and aggregation of ovine prion protein associated with classical and atypical scrapie correlates with the ease of unwinding of helix-2
    Tim J Fitzmaurice
    Department of Veterinary Medicine, University of Cambridge, Madingley Road, Cambridge CB3 OES, UK
    Biochem J 409:367-75
    ..to scrapie disease in sheep, the archetypal prion disease, correlates with polymorphisms within the ovine PrP (prion-related protein) gene...
  13. Copper-induced structural changes in the ovine prion protein are influenced by a polymorphism at codon 112
    Sujeong Yang
    Department of Veterinary Medicine, University of Cambridge, Madingley Road, Cambridge, CB3 OES, UK
    Biochim Biophys Acta 1784:683-92
    Prion diseases are associated with conformational change in the copper-binding protein PrP. The copper-binding sites in PrP are located in the N-terminal region of the molecule and comprise a series of tandem repeats of the sequence ..
  14. Frequencies of PrP genotypes and their implication for breeding against scrapie susceptibility in nine Pakistani sheep breeds
    M E Babar
    Molecular Cytogenetics and Genomics Laboratory, University of Veterinary and Animal Sciences, Lahore, Pakistan
    Mol Biol Rep 36:561-5
    Prion protein (PrP) gene of 308 sheep was genotyped to investigate polymorphisms at scrapie-associated codons 136, 154 and 171 to assess the resistance of nine different Pakistani sheep breeds to natural/typical scrapie...
  15. Strain-associated variations in abnormal PrP trafficking of sheep scrapie
    Martin Jeffrey
    Veterinary Laboratories Agency Lasswade, Pentlands Science Park, Bush Loan, Penicuik, Midlothian, Edinburgh, UK
    Brain Pathol 19:1-11
    Prion diseases are associated with the accumulation of an abnormal form of the host-coded prion protein (PrP)...
  16. Polymorphisms of the prion protein gene in sheep of Inner Mongolia, China
    Yiqin Wang
    National Animal Transmissible Spongiform Encephalopathies Laboratory, College of Veterinary Medicine, China Agricultural University, Beijing 100094, China
    Virus Genes 37:128-30
    Polymorphisms of the prion protein gene (Prnp), especially the amino acid residue alterations at codons 136, 154, and 174, in sheep have been found to be associated with susceptibility to scrapie disease...
  17. Neuroanatomical distribution of abnormal prion protein in naturally occurring atypical scrapie cases in Great Britain
    Sarah Jo Moore
    Department of Pathology, VLA Weybridge, New Haw, Addlestone, Surrey, KT15 3NB, UK
    Acta Neuropathol 116:547-59
    ..We found that the distribution of disease-associated prion protein (PrP(Sc)) and vacuolation in atypical scrapie cases are very different to both classical scrapie and experimental bovine ..
  18. Impact of strong selection for the PrP major gene on genetic variability of four French sheep breeds(Open Access publication)
    Isabelle Palhiere
    INRA, UR631 Station d Amélioration Génétique des Animaux, 31326 Castanet Tolosan, France
    Genet Sel Evol 40:663-80
    Effective selection on the PrP gene has been implemented since October 2001 in all French sheep breeds. After four years, the ARR "resistant" allele frequency increased by about 35% in young males...
  19. The epididymal soluble prion protein forms a high-molecular-mass complex in association with hydrophobic proteins
    Heath Ecroyd
    Gamète Male et Fertilité, Institut National de la Recherche Agronomique, INRA Nouzilly, 37380 Monnaie, France
    Biochem J 392:211-9
    We have shown previously that a 'soluble' form of PrP (prion protein), not associated with membranous vesicles, exists in the male reproductive fluid [Ecroyd, Sarradin, Dacheux and Gatti (2004) Biol. Reprod. 71, 993-1001]...
  20. Prion protein NMR structures of cats, dogs, pigs, and sheep
    Dominikus A Lysek
    Institut für Molekularbiologie und Biophysik, Eidgenössische Technische Hochschule Zürich, CH 8093 Zürich, Switzerland
    Proc Natl Acad Sci U S A 102:640-5
    The NMR structures of the recombinant cellular form of the prion proteins (PrPC) of the cat (Felis catus), dog (Canis familiaris), and pig (Sus scrofa), and of two polymorphic forms of the prion protein from sheep (Ovis aries) are ..
  21. Two alleles of a neural protein gene linked to scrapie in sheep
    W Goldmann
    Center for Molecular Biology, University of Heidelberg, Federal Republic of Germany
    Proc Natl Acad Sci U S A 87:2476-80
    ..Scrapie-associated fibrils [and their major protein, prion protein (PrP)] accumulate in the brains of all species affected by scrapie and related diseases...
  22. Identification of five allelic variants of the sheep PrP gene and their association with natural scrapie
    P B Belt
    Department of Molecular Biology, DLO Institute for Animal Science and Health ID DLO, Lelystad, The Netherlands
    J Gen Virol 76:509-17
    ..The host encoded prion protein (PrP) plays a central role in the disease process...
  23. Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie
    D Westaway
    Department of Neurology, University of California, San Francisco 94143
    Genes Dev 8:959-69
    ..the development of natural scrapie, echo reports of recessive manifestation, and parallel over-representation of PRNP codon 129 homozygotes in Creutzfeldt-Jakob disease of humans...
  24. PrP polymorphisms associated with natural scrapie discovered by denaturing gradient gel electrophoresis
    J L Laplanche
    FRA C Bernard Neurochimie des Communications Cellulaires, Hôpital Saint Louis, Paris, France
    Genomics 15:30-7
    ..An abnormal protease-resistant form of the host-encoded prion protein (PrP) accumulates in the brains of affected animals...
  25. PrP genotype contributes to determining survival times of sheep with natural scrapie
    A Bossers
    Department of Bacteriology, DLO Institute for Animal Science and Health, Lelystad, The Netherlands
    J Gen Virol 77:2669-73
    Several allelic variants of the sheep PrP gene are associated with scrapie susceptibility. However, it is not known whether, and to what extent, the PrP genotype contributes to determining survival times of scrapie sheep...
  26. Complete genomic sequence and analysis of the prion protein gene region from three mammalian species
    I Y Lee
    Department of Molecular Biotechnology, University of Washington, Seattle, Washington 98195 7730 USA
    Genome Res 8:1022-37
    The prion protein (PrP), first identified in scrapie-infected rodents, is encoded by a single exon of a single-copy chromosomal gene. In addition to the protein-coding exon, PrP genes in mammals contain one or two 5'-noncoding exons...
  27. PrP (prion) gene expression in sheep may be modulated by alternative polyadenylation of its messenger RNA
    W Goldmann
    Neuropathogenesis Unit, BBSRC Institute for Animal Health, Edinburgh, UK
    J Gen Virol 80:2275-83
    Scrapie-associated fibrils and their major protein component, PrP or prion protein, accumulate in the brains and some other tissues of all species affected by transmissible spongiform encephalopathies or prion diseases...
  28. Preclinical diagnosis of scrapie by immunohistochemistry of third eyelid lymphoid tissue
    T V Baszler
    Animal Disease Research Unit, Animal Research Service, U S Department of Agriculture, College of Veterinary Medicine, Washington State University, Pullman 99164 6630, USA
    J Clin Microbiol 38:3254-9
    ..family of fatal neurologic disorders characterized by deposition of an abnormal isoform (prion protein [PrP] PrP-Sc) of a cellular sialoglycoprotein in neural tissue...
  29. Plasminogen binds to disease-associated prion protein of multiple species
    M Maissen
    Lancet 357:2026-8
  30. PrP genotype in Sarda breed sheep and its relevance to scrapie. Brief report
    G Vaccari
    Istituto Zooprofilattico Sperimentale delle Regioni Lazio e Toscana, Rome, Italy
    Arch Virol 146:2029-37
    Several PrP gene polymorphisms modulate sheep scrapie susceptibility. Recently, an increase of scrapie outbreaks has been reported in Italy. A vaccine containing sheep brain homogenate was used in most of the outbreaks...
  31. Prion protein gene polymorphisms in healthy and scrapie-affected sheep in Greece
    Charalambos Billinis
    Laboratory of Microbiology and Infectious Diseases, Faculty of Veterinary Medicine, Aristotle University, GR 54124 Thessaloniki, Greece
    J Gen Virol 85:547-54
    ..Chios and Karagouniko from healthy flocks were analysed for scrapie-linked polymorphisms in the prion protein (PrP) gene...
  32. Identification of a novel ovine PrP polymorphism and scrapie-resistant genotypes for St. Croix White and a related composite breed
    C M Seabury
    Department of Veterinary Pathobiology, College of Veterinary Medicine, Texas A and M University, College Station, TX 77843 4467, USA
    Cytogenet Genome Res 102:85-8
    Susceptibility to scrapie is primarily controlled by polymorphisms in the ovine prion protein gene (PRNP)...
  33. The crystal structure of the globular domain of sheep prion protein
    L F Haire
    Structural Biology Group, Division of Protein Structure, National Institute for Medical Research, The Ridgeway, Mill Hill, London NW7 1AA, UK
    J Mol Biol 336:1175-83
    ..These loci provide sites within the PrPc monomer that could readily give rise to early intermediate species on the pathway to the formation of aggregated ..
  34. 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
    Bryan Maloney
    Departments of Psychiatry, Institute of Psychiatric Research, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA
    FASEB J 18:1288-90
    ....
  35. Characterization of a complex formed between human plasminogen and recombinant sheep prion: pressure and thermal sensitivity of complex formation
    J A Kornblatt
    Concordia University, Montreal, QC, Canada
    Cell Mol Biol (Noisy-le-grand) 50:387-96
    ..as the scrapie form of the prion protein (PrPsc) whereas a benign form, the cellular conformer, is referred to as PrPC. The cellular conformation of the sheep prion protein formed a 1:1 complex with human plasminogen...
  36. Positive correlation between relative mRNA expression of PRNP and SPRN in cerebral and cerebellar cortex of sheep
    Evelyne Lampo
    Department of Nutrition, Faculty of Veterinary Medicine, Ghent University, Heidestraat 19, B 9820 Merelbeke, Belgium
    Mol Cell Probes 23:60-4
    SPRN is an interesting new member of the PRNP family because of its sequence homology with the hydrophobic region of PRNP, its expression in brain tissue and its PrP-like properties in functional experiments on Prnp(0/0) mice...