Prion Protein (PRNP)

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CD230; GSS; ASCR; CJD; PRIP; PrP; PrP2730; PrPc; Major prion protein; Creutzfeldt-Jakob Disease; Gerstmann-Strausler-Scheinker Syndrome; Fatal Familial Insomnia

Prion Protein (PRNP)
  • PRNP is a gene that codes for a protein called the prion protein (PrP), which is expressed in the brain and several other tissues.The human PRNP gene is located on the short (p) arm of chromosome 20 between the end (terminus) of the arm and position 12, from base pair 4,615,068 to base pair 4,630,233. PRNP has also recently been designated CD230 (cluster of differentiation 230).

  • Although the precise function of PrP is not yet known, it is possibly involved in the transport of ionic copper to cells from the surrounding environment. Researchers have also proposed roles for PrP in cell signaling or in the formation of gaps between nerve cells (synapses) where cell-to-cell communication occurs.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPB680Hu01 Recombinant Prion Protein (PRNP) Positive Control; Immunogen; SDS-PAGE; WB.
EPB680Hu61 Eukaryotic Prion Protein (PRNP) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAB680Hu01 Polyclonal Antibody to Prion Protein (PRNP) WB; IHC; ICC; IP.
MAB680Hu21 Monoclonal Antibody to Prion Protein (PRNP) WB; IHC; ICC; IP.
PAB680Hu02 Polyclonal Antibody to Prion Protein (PRNP) WB; IHC; ICC; IP.
Assay Kits SEB680Hu ELISA Kit for Prion Protein (PRNP) Enzyme-linked immunosorbent assay for Antigen Detection.
HEB680Hu High Sensitive ELISA Kit for Prion Protein (PRNP) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPB680Mu01 Recombinant Prion Protein (PRNP) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAB680Mu01 Polyclonal Antibody to Prion Protein (PRNP) WB; IHC; ICC; IP.
Assay Kits SEB680Mu ELISA Kit for Prion Protein (PRNP) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Prion Protein (PRNP) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Prion Protein (PRNP) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Prion Protein (PRNP) Polyclonal Antibody Customized Service Offer
Assay Kits SEB680Ra ELISA Kit for Prion Protein (PRNP) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Bos taurus; Bovine (Cattle)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Prion Protein (PRNP) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Prion Protein (PRNP) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Prion Protein (PRNP) Polyclonal Antibody Customized Service Offer
Assay Kits SEB680Bo ELISA Kit for Prion Protein (PRNP) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Chicken (Gallus)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Prion Protein (PRNP) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Prion Protein (PRNP) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Prion Protein (PRNP) Polyclonal Antibody Customized Service Offer
Assay Kits SEB680Ga ELISA Kit for Prion Protein (PRNP) Enzyme-linked immunosorbent assay for Antigen Detection.
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  2. "Genomic structure of the human prion protein gene."Am. J. Hum. Genet. 49:320-329(1991) [PubMed] [Europe PMC] [Abstract]
  3. "Complete genomic sequence and analysis of the prion protein gene region from three mammalian species."Genome Res. 8:1022-1037(1998) [PubMed] [Europe PMC] [Abstract]
  4. "Novel PRNP sequence variant associated with familial encephalopathy."Am. J. Med. Genet. 88:653-656(1999) [PubMed] [Europe PMC] [Abstract]
  5. "The DNA sequence and comparative analysis of human chromosome 20." Nature 414:865-871(2001) [PubMed] [Europe PMC] [Abstract]
  6. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
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  8. "Deletion in the prion protein gene in a demented patient."Hum. Mol. Genet. 1:443-444(1992) [PubMed] [Europe PMC] [Abstract]
  9. "A prion-linked psychiatric disorder."Nature 390:241-241(1997) [PubMed] [Europe PMC] [Abstract]
  10. "SSCP analysis and sequencing of the human prion protein gene (PRNP) detects two different 24 bp deletions in an atypical Alzheimer's disease family."Am. J. Med. Genet. 60:12-18(1995) [PubMed] [Europe PMC] [Abstract]
  11. "Amyloid protein of Gerstmann-Straussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58."EMBO J. 10:513-519(1991) [PubMed] [Europe PMC] [Abstract]
  12. "Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene."Proc. Natl. Acad. Sci. U.S.A. 88:10926-10930(1991) [PubMed] [Europe PMC] [Abstract]
  13. "Prion, amyloid beta-derived Cu(II) ions, or free Zn(II) ions support S-nitroso-dependent autocleavage of glypican-1 heparan sulfate."J. Biol. Chem. 278:38956-38965(2003) [PubMed] [Europe PMC] [Abstract]
  14. "The octarepeat domain of the prion protein binds Cu(II) with three distinct coordination modes at pH 7.4."J. Am. Chem. Soc. 127:12647-12656(2005) [PubMed] [Europe PMC] [Abstract]
  15. "The prion protein is a combined zinc and copper binding protein: Zn2+ alters the distribution of Cu2+ coordination modes."J. Am. Chem. Soc. 129:15440-15441(2007) [PubMed] [Europe PMC] [Abstract]
  16. "Biosynthesis of prion protein nucleocytoplasmic isoforms by alternative initiation of translation."J. Biol. Chem. 284:2787-2794(2009) [PubMed] [Europe PMC] [Abstract]
  17. "Mass-spectrometric identification and relative quantification of N-linked cell surface glycoproteins."Nat. Biotechnol. 27:378-386(2009) [PubMed] [Europe PMC] [Abstract]
  18. "Glypican-1 mediates both prion protein lipid raft association and disease isoform formation."PLoS Pathog. 5:E1000666-E1000666(2009) [PubMed] [Europe PMC] [Abstract]
  19. "Early onset prion disease from octarepeat expansion correlates with copper or zinc binding properties."PLoS Pathog. 5:E1000390-E1000390(2009) [PubMed] [Europe PMC] [Abstract]
  20. "Prion fibrillization is mediated by a native structural element that comprises helices H2 and H3."J. Biol. Chem. 285:21004-21012(2010) [PubMed] [Europe PMC] [Abstract]
  21. "Copper (II) promotes the formation of soluble neurotoxic PrP oligomers in acidic environment."J. Cell. Biochem. 111:627-633(2010) [PubMed] [Europe PMC] [Abstract]
  22. "Characterizing the novel protein p33MONOX."Mol. Cell. Biochem. 350:127-134(2011) [PubMed] [Europe PMC] [Abstract]
  23. "Solution structure of the E200K variant of human prion protein. Implications for the mechanism of pathogenesis in familial prion diseases."J. Biol. Chem. 275:33650-33654(2000) [PubMed] [Europe PMC] [Abstract]
  24. "NMR solution structure of the human prion protein."Proc. Natl. Acad. Sci. U.S.A. 97:145-150(2000) [PubMed] [Europe PMC] [Abstract]
  25. "NMR structures of three single-residue variants of the human prion protein."Proc. Natl. Acad. Sci. U.S.A. 97:8340-8345(2000) [PubMed] [Europe PMC] [Abstract]
  26. "Crystal structure of the human prion protein reveals a mechanism for oligomerization."Nat. Struct. Biol. 8:770-774(2001) [PubMed] [Europe PMC] [Abstract]
  27. "Molecular features of the copper binding sites in the octarepeat domain of the prion protein."Biochemistry 41:3991-4001(2002) [PubMed] [Europe PMC] [Abstract]
  28. "The octapeptide repeats in mammalian prion protein constitute a pH-dependent folding and aggregation site."J. Mol. Biol. 334:477-488(2003) [PubMed] [Europe PMC] [Abstract]
  29. "Mutations and polymorphisms in the prion protein gene."Hum. Mutat. 2:168-173(1993) [PubMed] [Europe PMC] [Abstract]
  30. "Genetic and infectious prion diseases."Arch. Neurol. 50:1129-1153(1993) [PubMed] [Europe PMC] [Abstract]
  31. "Atomic structures of amyloid cross-beta spines reveal varied steric zippers."Nature 447:453-457(2007) [PubMed] [Europe PMC] [Abstract]
  32. "Crystal structure of human prion protein bound to a therapeutic antibody."Proc. Natl. Acad. Sci. U.S.A. 106:2554-2558(2009) [PubMed] [Europe PMC] [Abstract]
  33. "Conformational diversity in prion protein variants influences intermolecular beta-sheet formation."EMBO J. 29:251-262(2010) [PubMed] [Europe PMC] [Abstract]
  34. "Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome."Nature 338:342-345(1989) [PubMed] [Europe PMC] [Abstract]
  35. "Pro-->Leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Straussler syndrome."Biochem. Biophys. Res. Commun. 163:974-979(1989) [PubMed] [Europe PMC] [Abstract]
  36. "Fatal familial insomnia: a second kindred with mutation of prion protein gene at codon 178."Neurology 42:669-670(1992) [PubMed] [Europe PMC] [Abstract]
  37. "New mutation in scrapie amyloid precursor gene (at codon 178) in Finnish Creutzfeldt-Jakob kindred."Lancet 337:425-425(1991) [PubMed] [Europe PMC] [Abstract]
  38. "Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia."Lancet 336:514-515(1990) [PubMed] [Europe PMC] [Abstract]
  39. "Mutant prion proteins in Gerstmann-Straussler-Scheinker disease with neurofibrillary tangles."Nat. Genet. 1:68-71(1992) [PubMed] [Europe PMC] [Abstract]
  40. "Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Straussler syndrome."Biochem. Biophys. Res. Commun. 191:709-714(1993) [PubMed] [Europe PMC] [Abstract]
  41. "A new point mutation of the prion protein gene in Creutzfeldt-Jakob disease."Ann. Neurol. 34:802-807(1993) [PubMed] [Europe PMC] [Abstract]
  42. "A missense mutation at codon 105 with codon 129 polymorphism of the prion protein gene in a new variant of Gerstmann-Straussler-Scheinker disease."Neurology 43:2723-2724(1993) [PubMed] [Europe PMC] [Abstract]
  43. "A variant of Gerstmann-Straussler-Scheinker disease carrying codon 105 mutation with codon 129 polymorphism of the prion protein gene: a clinicopathological study."J. Neurol. Sci. 127:77-86(1994) [PubMed] [Europe PMC] [Abstract]
  44. "Japanese family with Creutzfeldt-Jakob disease with codon 200 point mutation of the prion protein gene."Neurology 44:299-301(1994) [PubMed] [Europe PMC] [Abstract]
  45. "Mutation in codon 200 and polymorphism in codon 129 of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease."Philos. Trans. R. Soc. Lond., B, Biol. Sci. 343:385-390(1994) [PubMed] [Europe PMC] [Abstract]
  46. "Gerstmann-Straussler-Scheinker disease with mutation at codon 102 and methionine at codon 129 of PRNP in previously unreported patients."Neurology 45:1127-1134(1995) [PubMed] [Europe PMC] [Abstract]
  47. "Polymorphism at codon 129 or codon 219 of PRNP and clinical heterogeneity in a previously unreported family with Gerstmann-Straussler-Scheinker disease (PrP-P102L mutation)."Neurology 47:734-741(1996) [PubMed] [Europe PMC] [Abstract]
  48. "Mutation of the prion protein gene at codon 208 in familial Creutzfeldt-Jakob disease."Neurology 47:1305-1312(1996) [PubMed] [Europe PMC] [Abstract]
  49. "Familial spongiform encephalopathy associated with a novel prion protein gene mutation."Ann. Neurol. 42:138-146(1997) [PubMed] [Europe PMC] [Abstract]
  50. "Phenotypic variability of Gerstmann-Straussler-Scheinker disease is associated with prion protein heterogeneity." J. Neuropathol. Exp. Neurol. 57:979-988(1998) [PubMed] [Europe PMC] [Abstract]
  51. "Molecular genetics of human prion diseases in Germany."Hum. Genet. 105:244-252(1999) [PubMed] [Europe PMC] [Abstract]
  52. "High prevalence of pathogenic mutations in patients with early-onset dementia detected by sequence analyses of four different genes."Am. J. Hum. Genet. 66:110-117(2000) [PubMed] [Europe PMC] [Abstract]
  53. "Identification of three novel mutations (E196K, V203I, E211Q) in the prion protein gene (PRNP) in inherited prion diseases with Creutzfeldt-Jakob disease phenotype."Hum. Mutat. 15:482-482(2000) [PubMed] [Europe PMC] [Abstract]
  54. "A new PRNP mutation (G131V) associated with Gerstmann-Straussler-Scheinker disease."Arch. Neurol. 58:1899-1902(2001) [PubMed] [Europe PMC] [Abstract]