Keywords: PrP، پروتئین پریون; CD, circular dichroism; GPI, glycosylphosphatidylinositol; FTIR, Fourier transform infrared; ITC, isothermal titration calorimetry; PrP, prion protein; PrPΔocta, human prion protein with the deletion of octarepeats (58–89); PrPC, the normal cellular PrP m
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The pathogenesis of soluble PrP fragments containing Aβ binding sites
Keywords: PrP، پروتئین پریون; PD, prion diseases; AD, Alzheimer’s disease; PrP, prion protein; GSS, Gerstmann–Sträussler–Scheinker; GPI, glycosyl-phosphatidylinositol; APP, amyloid precursor protein; LTP, long-term potentiation; CJD, Creutzfeldt–Jakob diseaseSoluble PrP95-105; Amyloid
A single amino acid (Asp159) from the dog prion protein suppresses the toxicity of the mouse prion protein in Drosophila
Keywords: PrP، پروتئین پریون; α1–3, helices 1–3; CaPrP, Canis PrP; MoPrP, mouse PrP; PrP, prion protein; WT, wild typePrion protein; Neurotoxicity; Dog; Mouse; Drosophila; Misfolding; Protective substitution; Sequence alignment
Human Doppel and prion protein share common membrane microdomains and internalization pathways
Keywords: PrP، پروتئین پریون; Dpl, Doppel; PrP, prion protein; PrPc, cellular prion protein; Prnd, Dpl encoding gene; Prnp, PrP encoding gene; GPI, glycosylphosphatidylinositol; GFP, green fluorescent protein; CFP, cyan fluorescent protein; YFP, yellow fluorescent protein; CNTF, cilia
Mutant PrPCJD prevails over wild-type PrPCJD in the brain of V210I and R208H genetic Creutzfeldt–Jakob disease patients
Keywords: PrP، پروتئین پریون; CJD, Creutzfeldt–Jakob disease; PrP, prion protein; TSEs, transmissible spongiform encephalopathies; GSS, Gerstmann–Sträussler–Scheinker disease; PRNP, PrP gene; SDS–PAGE, sodium dodecyl sulphate polyacrylamide gel electrophoresis; DTT, dithiothreitol; CN
Frameshifted prion proteins as pathological agents: Quantitative considerations
Keywords: PrP، پروتئین پریون; TSE, transmissible spongiform encephalopathy; PrP, prion protein; PMCA, protein misfolding cyclic amplification; AltPrP, alternative prion protein; PRF, programmed ribosomal frameshift; TAR, trans-acting response; HIV, human immunodeficiency virus; eIF, e
Cloning and polymorphism analysis of prion protein gene in domestic bactrian camel in China
Keywords: PrP، پروتئین پریون; TSE, transmissible spongiform encephalopathies; PrP, Prion protein; PRNP, prion protein gene; PrPc, normal cellular PrP; PrPsc, scrapie isoform of PrP; BSE, bovine spongiphorm encephalopathy; PCR, polyenzyme chain reaction; ORF, open reading frame; GPI, g
Novel assay with fluorescence-labelled PrP peptides for differentiating L-type atypical and classical BSEs, and scrapie
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrPC, cellular prion protein; PrPSc, disease-associated prion protein; BSE, bovine spongiform encephalopathy; C-BSE, classical BSE; L-BSE, L-type atypical BSE; PK, proteinase K; FI, fluorescence indexPrion; PrPSc; Conformational change
Coordination of zinc ions to the key proteins of neurodegenerative diseases: Aβ, APP, α-synuclein and PrP
Keywords: PrP، پروتئین پریون; AD, Alzheimer's disease; PD, Parkinson's disease; TSE, transferable spongiform encephalitis; APP, amyloid-β precursor protein; Aβ, amyloid-β peptide; PrP, prion protein; CuBD, copper binding domainZinc; Alzheimer's disease; Parkinson disease; Prion; Amylo
Atorvastatin stimulates neuroblastoma cells to induce neurite outgrowth by increasing cellular prion protein expression
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrPC, cellular prion protein; PrPSc, scrapie prion protein; AD, Alzheimer's diseasePrion protein; HMG-CoA reductase inhibitor; Neurite outgrowth; Neuro2a cells
Genetically engineered mouse models of the trinucleotide-repeat spinocerebellar ataxias
Keywords: PrP، پروتئین پریون; A, alanine; BAC, bacterial artificial chromosome; NII, neuronal intranuclear inclusion; PC, Purkinje cell; PrP, prion protein; Q, glutamine; RORα, retinoid-related orphan receptor-alpha; S, serine; SCA, spinocerebellar ataxia; Tet, tetracycline; Tg, trans
Metal ions and amyloid fiber formation in neurodegenerative diseases. Copper, zinc and iron in Alzheimer's, Parkinson's and prion diseases
Keywords: PrP، پروتئین پریون; Aβ, amyloid-β-peptide; AD, Alzheimer's disease; CD, circular dichroism; NMDAR, N-methyl-d-aspartate receptors; PD, Parkinson's disease; PrP, prion protein; αSyn, α-synuclein; ThT, thioflavin T; TSEs, transmissible spongiform encephalopathies; TEM, transmi
Epitope mapping of a PrP(Sc)-specific monoclonal antibody: Identification of a novel C-terminally truncated prion fragment
Keywords: PrP، پروتئین پریون; (s)CJD, (sporadic) Creutzfeldt-Jakob disease; HRP, horseradish peroxidase; HuPrP, human prion protein; KLH, keyhole limpet haemocyanin; LB, Luria broth; mAb, monoclonal antibody; PK, proteinase K; PrP, prion protein; PrPC, cellular prion protein; PrPres,
Heparin enhances the cell-protein misfolding cyclic amplification efficiency of variant Creutzfeldt–Jakob disease
Keywords: PrP، پروتئین پریون; BSE, bovine spongiform encephalopathy; HMW, high molecular weight; LMW, low molecular weight; MW, molecular weight; PMCA, protein misfolding cyclic amplification; PPS, pentosan polysulfate; PrP, prion protein; sCJD, sporadic Creutzfeldt–Jakob disease; vCJ
Aged PrP null mice show defective processing of neuregulins in the peripheral nervous system
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrPC, cellular form of the PrP; TSE, transmissible spongiform encephalopathy; APP, amyloid precursor protein; BACE1, β-site of APP cleaving enzyme-1; NRG, neuregulin protein family; NRG1, neuregulin-1 proteins; NRG1-FL, full length neu
Dynamics of polymerization shed light on the mechanisms that lead to multiple amyloid structures of the prion protein
Keywords: PrP، پروتئین پریون; ATF, Atomic Force; CD, Circular Dichroism; FACS, Fluorescence Activated Cells Sorting; GdnCl, Guanidium Chloride; PrP, Prion protein; rPrP, recombinant prion protein; FTIR, Fourier-transform infrared spectroscopy; ThT, Thioflavin-TPrion; Amyloid; Heteroge
Fish models in prion biology: Underwater issues
Keywords: PrP، پروتئین پریون; TSEs, transmissible spongiform encephalopathies; PrP, prion protein; BSE, bovine spongiform encephalopathy; CJD, Creutzfeldt–Jakob disease; GPI, glycosylphospatidylinositol; MBM, meat and bone meal; p.i., post inoculation; i.c., intracerebrally; PK, prote
Copper(II)-Induced Secondary Structure Changes and Reduced Folding Stability of the Prion Protein
Keywords: PrP، پروتئین پریون; PrP, prion protein; GdmCl, guanidinium chloride; mPrP, mouse PrP; FTIR, Fourier transform infrared spectroscopyCu2+; urea; unfolding; PrP; circular dichroism
A radiation-induced adaptive response prolongs the survival of prion-infected mice
Keywords: PrP، پروتئین پریون; PrP, prion protein; ROS, reactive oxygen species; dpi, days postinfection; 8-OHdG, 8-hydroxydeoxyguanosine; HSF-1, heat shock factor 1Prion; Low-dose irradiation; Oxidative stress; Adaptive response; ME-7; Mice; Neurodegeneration; Free radicals
The depletion of α and β PrP from complex mixtures
Keywords: PrP، پروتئین پریون; IMAC, immobilised metal ion affinity chromatography; PrP, prion protein; PrPC, endogenous prion protein; PrPSc, infectious prion proteinPrions; Ferric compounds; Iron; Isolation and purification; Peptide library; Phage display
The Unfolded State of the Murine Prion Protein and Properties of Single-point Mutants Related to Human Prion Diseases
Keywords: PrP، پروتئین پریون; PrP, prion protein; hPrP, human prion protein; mPrP, mouse prion protein; (PrPC), cellular form of PrP; PrPSc, “scrapie” form of PrP; mPrPred, the prion protein from mouse, mPrP(121−232), in the reduced state; mPrPox, the prion protein from mouse, mPrP(12
Horse Prion Protein NMR Structure and Comparisons with Related Variants of the Mouse Prion Protein
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrPC, cellular form of PrP; PrPSc, scrapie form of PrP; ecPrP, horse PrP; mPrP, mouse PrP; TSE, transmissible spongiform encephalopathy; NOE, nuclear Overhauser enhancement; NOESY, NOE spectroscopy; ePrP, elk PrP; bvPrP, bank vole PrPc
Prion Interaction with the 37-kDa/67-kDa Laminin Receptor on Enterocytes as a Cellular Model for Intestinal Uptake of Prions
Keywords: PrP، پروتئین پریون; CWD, chronic wasting disease; BSE, bovine spongiform encephalopathy; TSE, transmissible spongiform encephalopathy; PrP, prion protein; FACS, fluorescence-activated cell scanning; 2D, two-dimensional; PBS, phosphate-buffered saline; FCS, fetal calf serum;
Genomic assessment of the evolution of the prion protein gene family in vertebrates
Keywords: PrP، پروتئین پریون; CJD, Creutzfedlt-Jakob disease; BSE, bovine spongiform encepahalopathy; TSE, transmissible spongiform encephalopathy; PrP, prion protein; NMR, nuclear magnetic resonance; CNS, central nervous system; TM, transmembrane; PrP-GF, PrP gene family; GPI, glycos
The Common Architecture of Cross-β Amyloid
Keywords: PrP، پروتئین پریون; ssNMR, solid-state nuclear magnetic resonance; IAPP, islet amyloid polypeptide; TTR, transthyretin; β2m, β2-microglobulin; PrP, prion protein; SOD1, superoxide dismutase 1amyloid; X-ray fibre diffraction; fibril structure; cross-β; β-helix
Vesicle Permeabilization by Purified Soluble Oligomers of Prion Protein: A Comparative Study of the Interaction of Oligomers and Monomers with Lipid Membranes
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrPSc, scrapie PrP; PK, proteinase K; Aβ, amyloid β; IAPP, islet amyloid polypeptide; CF, carboxyfluorescein; SEC, size-exclusion chromatography; L/P, lipid-to-protein; DLS, dynamic light scattering; FTIR, Fourier transform infrared sp
Structure of the Flexible Amino-Terminal Domain of Prion Protein Bound to a Sulfated Glycan
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrP 23–106, PrP residues 23 to 106; PPS, pentosan polysulfate; 3D, three-dimensional; PrPsen, protease-sensitive PrP; TSE, transmissible spongiform encephalopathy; PrPres, protease-resistant PrP; PPII, polyproline II; HSQC, heteronucle
Pathogenic Mutations in the Hydrophobic Core of the Human Prion Protein Can Promote Structural Instability and Misfolding
Keywords: PrP، پروتئین پریون; PrP, prion protein; recPrP, recombinant prion protein; CJD, Creutzfeldt-Jakob disease; GPI, glycosylphosphatidyl-inositol; MD, molecular dynamics; WT, wild type; Cα RMSD, root-mean-square deviation of Cα atom coordinates from the starting structure; Cα RM
Cold destabilization and temperature jump of the murine prion protein mPrP(23–231)
Keywords: PrP، پروتئین پریون; PrP, prion protein; ID50, median infectious dose; cT-jump, continuous-wave probed laser-induced temperature jump; CD, circular dichroism; NATA, N-acetyltryptophanamidePrion protein; Folding intermediate; Cold denaturation; Structural stability; Laser-indu
Regulation of focal adhesion formation and filopodia extension by the cellular prion protein (PrPC)
Keywords: PrP، پروتئین پریون; PrP, prion protein; FA, focal adhesion; FAK, focal adhesion kinase; TIRFM, total internal reflection fluorescence microscopy; R1EA, reggie-1 EA construct; siRNA, small interfering RNAPrPC; Cell–substrate interaction; Filopodia/lamellipodia; Focal adhesion
Phospholipids influence the aggregation of recombinant ovine prions: From rapid extensive aggregation to amyloidogenic conversion
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrPC, cellular isoform of PrP; PrPSc, scrapie isoform of PrP; PrP, recombinant ovine PrP; GPI, glycosylphosphatidylinositol; PI, phosphatidylinositol; MG, 1-oleoylglycerol; TG, 1,2,3-trioleoylglycerol; PA, phosphatidic acid; PC, phosph
UV-light-induced conversion and aggregation of prion proteins
Keywords: PrP، پروتئین پریون; TSE, transmissible spongiform encephalopathy; PrP, prion protein; PrPC, cellular PrP; PrPSc, scrapie-like misfolded PrP; ROS, reactive oxygen species; CD, circular dichroism; DLS, dynamic light scattering; UV, ultraviolet; T1/2, time until the value reach
Association of a GPI-anchored protein with detergent-resistant membranes facilitates its trafficking through the early secretory pathway
Keywords: PrP، پروتئین پریون; BFA, Brefeldin A; DMEM, Dulbecco's Modified Eagle Medium; DRMs, detergent-resistant membranes; ER, endoplasmic reticulum; GPI, glycosylphosphatidylinositol; MDCK, Madin Darby canine kidney; MDP, membrane dipeptidase; PrP, prion protein; TGN, trans-Golgi n
Structural Insights into Alternate Aggregated Prion Protein Forms
Keywords: PrP، پروتئین پریون; rec, recombinant; PrP, prion protein; PrPC, cellular form of prion protein; PrPSc, scrapie prion protein; ASA, amyloid seeding assay; GdnHCl, guanidine hydrochloride; ThT, thioflavin T; PTA, phosphotungstate; AFM, atomic force microscopyprion protein (PrP
Peptide NMHRYPNQ of the Cellular Prion Protein (PrPC) Inhibits Aggregation and Is a Potential Key for Understanding Prion–Prion Interactions
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrPC, cellular PrP; PrPSc, scrapie form of PrP; hPrP, human PrP; rhPrP, recombinant hPrP; CJD, Creutzfeldt–Jakob disease; SPR, surface plasmon resonance; STD, saturation transfer difference; TSE, transmissible spongiform encephalopathy
Effect of enzymatic deimination on the conformation of recombinant prion protein
Keywords: PrP، پروتئین پریون; BSE, Bovine Spongiform Encephalopathy; CJD, Creutzfeldt–Jakob disease; PrP, prion protein; PrPC, cellular form of PrP; PrPSc, scrapie (pathological) form of PrP; huPrP, human PrP; ovPrP, ovine PrP; recPrP, recombinant PrP; PAD, peptidylarginine deiminase;
A large ribonucleoprotein particle induced by cytoplasmic PrP shares striking similarities with the chromatoid body, an RNA granule predicted to function in posttranscriptional gene regulation
Keywords: PrP، پروتئین پریون; CyPrPEGFP, cytoplasmic prion protein genetically fused to EGFP; DDX6, DEAD-box RNA helicase 6; EGFP, enhanced green fluorescent protein; miRNA, microRNA; PrP, prion protein; PrP-RNP, cyPrP-induced RiboNucleoprotein Particle; SmB/B′/N, Small nuclear ribonu
Prion Protein NMR Structure from Tammar Wallaby (Macropus eugenii) Shows that the β2–α2 Loop Is Modulated by Long-Range Sequence Effects
Keywords: PrP، پروتئین پریون; COSY, correlation spectroscopy; HSQC, heteronuclear single quantum coherence; PrP, prion protein; PrPC, cellular isoform of PrP; PrPSc, scrapie isoform of PrPC; mPrP, mouse PrP; twPrP, tammar wallaby PrP; NOE, nuclear Overhauser enhancement; NOESY, nuclea
Subcellular localization of prion proteins and the 37 kDa/67 kDa laminin receptor fused to fluorescent proteins
Keywords: PrP، پروتئین پریون; LRP/LR, laminin receptor precursor/laminin receptor; EGFP, enhanced green fluorescent protein; ECFP, enhanced cyan fluorescent protein; PrP, prion protein; PrPc, cellular form of PrP; Prnp, PrP gene; ΔSP-PrP mutant, PrP mutant lacking its signal peptide;
Structure, dynamics, and stability of assemblies of the human prion fragment SNQNNF
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrPC, cellular prion protein; PrPSc, scrapie prion protein; RMSD, root mean square deviation; RMSF, root mean square fluctuation; PDB, Protein Data Bank; MD, molecular dynamicsPrion protein; Molecular dynamics; Steric zipper; Prion hyd
Relevance of the N-terminal NLS-like sequence of the prion protein for membrane perturbation effects
Keywords: PrP، پروتئین پریون; NLS, Nuclear Localization Sequence; PrP, Prion Protein; PrPC, cellular isoform of PrP; PrPSc, scrapie isoform of PrP; mPrPp(1–28), residues 1–28 of the mouse PrP sequence; mPrPp(23–50), residues 23–50 of the mouse PrP sequence; bPrPp(1–30), residues 1–30
Native, amyloid fibrils and β-oligomers of the C-terminal domain of human prion protein display differential activation of complement and bind C1q, factor H and C4b-binding protein directly
Keywords: PrP، پروتئین پریون; C4BP, C4b-binding protein; CRP, C-reactive protein; FDC, follicular dendritic cell; FH, factor H; MAC, membrane attack complex; MBL, mannose-binding lectin; NHS, normal human serum; PrP, prion protein; PrPC, prion protein—cellular variant; PrPSc, prion pr
Globular and pre-fibrillar prion aggregates are toxic to neuronal cells and perturb their electrophysiology
Keywords: PrP، پروتئین پریون; ATR FTIR, attenuated total reflection Fourier transform infrared; CD, circular dichroism; CNS, central nervous system; EM, electron microscopy; PrP, prion protein; MTT, 3-(4,5-dimethylthiazol-2-yl)-2, 5-diphenyltetrazolium bromide; SHaPrP, Syrian hamster
Opposing roles of prion protein in oxidative stress- and ER stress-induced apoptotic signaling
Keywords: PrP، پروتئین پریون; PrP, prion protein; BFA, brefeldin A; TUN, tunicamycin; Z-VAD-FMK, Z-Val-Ala-Asp-fluoromethyl ketone; Z-DEVD-FMK, Z-Asp-Glu-Val-Asp-fluoromethyl ketone; Z-IETD-FMK, Z-Ile-Glu-Thr-Asp-fluoromethyl ketone; Z-LEHD-FMK, Z-Leu-Glu-His-Asp-fluoromethyl ketone;
Prion protein aggresomes are poly(A)+ ribonucleoprotein complexes that induce a PKR-mediated deficient cell stress response
Keywords: PrP، پروتئین پریون; CyPrPEGFP, cytoplasmic prion protein genetically fused to EGFP; EGFP, enhanced green fluorescent protein; ER, endoplasmic reticulum; Hsp, heat shock protein; poly(I:C), polyinosinic–polycytidylic acid; PKR, RNA-dependent protein kinase; PrP, prion protein
The Polybasic N-Terminal Region of the Prion Protein Controls the Physical Properties of Both the Cellular and Fibrillar Forms of PrP
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrPC, cellular isoform of the prion protein; PrPSc, disease-associated isoform of the prion protein; PrP 23–231, full-length recombinant prion protein; PrP 31–231, recombinant prion protein encompassing residues 31–231; α-PrP, alpha-he
Phospholipid Interaction Induces Molecular-level Polymorphism in Apolipoprotein C-II Amyloid Fibrils via Alternative Assembly Pathways
Keywords: PrP، پروتئین پریون; PrP, prion protein; DnPC, 1,2-diacyl-sn-glycero-3-phosphocholine, where n = 3–9 carbon acyl chains; DMPC, 1,2-dimyristoyl-sn-glycero-3-phosphocholine; LysoMPC, 1-myristoyl-2-hydroxy-sn-glycero-3-phosphocholine; apo, apolipoprotein; CMC, critical micelle c
Newly established in vitro system with fluorescent proteins shows that abnormal expression of downstream prion protein-like protein in mice is probably due to functional disconnection between splicing and 3′ formation of prion protein pre-mRNA
Keywords: PrP، پروتئین پریون; PrP, prion protein; PrPLP/Dpl, PrP-like protein/Doppel; bp, base pair; PCR, polymerase chain reaction; nt, nucleotides; EGFP, enhanced green fluorescence protein; UTR, untranslated region; ORF, open reading frame; DMEM, Dulbecco's Modified Eagle Medium; R
Prion infection correlates with hypersensitivity of P2X7 nucleotide receptor in a mouse microglial cell line
Keywords: PrP، پروتئین پریون; PrP, prion protein; P2X7R, P2X7 receptor; IL-1β, interleukin-1β; PK, protenase K; LPS, lipopolysaccharide; BBG, brilliant blue-G; PPS, pentosan polysulfate; BSA, bovine serum albuminP2X7 receptor; ATP; Microglia; Prion; Cytosolic calcium
Cellular prion protein protects against reactive-oxygen-species-induced DNA damage
Keywords: PrP، پروتئین پریون; CJD, Creutzfeldt–Jakob disease; FPG, formamidopyrimidine DNA glycosylase; GSS, Gerstmann–-Sträussler–Scheinker; 8-OHG, 8-hydroxyguanosine; PBS, phosphate-buffered saline; PrP, prion protein; PrPC, cellular form of PrP; PrPSc, infectious, protease-resistan