Molecular advances in understanding inherited prion diseases

scientific article

Molecular advances in understanding inherited prion diseases is …
instance of (P31):
review articleQ7318358
scholarly articleQ13442814

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P6179Dimensions Publication ID1008605010
P356DOI10.1385/MN:25:3:287
P8608Fatcat IDrelease_tjm2qajfbbffjbnvcuk2xsiabe
P698PubMed publication ID12109876

P2093author name stringDavid R Brown
P2860cites workPrionsQ24633319
NMR solution structure of the human prion proteinQ27620845
Solution structure of the E200K variant of human prion protein. Implications for the mechanism of pathogenesis in familial prion diseasesQ27626645
Crystal structure of the human prion protein reveals a mechanism for oligomerizationQ27634441
NMR structure of the mouse prion protein domain PrP(121-231)Q27733163
Antioxidant activity related to copper binding of native prion proteinQ28141942
Prion diseases of humans and animals: their causes and molecular basisQ28209943
Mice devoid of PrP are resistant to scrapieQ28249108
Electron paramagnetic resonance evidence for binding of Cu(2+) to the C-terminal domain of the murine prion proteinQ28367705
Copper stimulates endocytosis of the prion proteinQ28589040
Novel proteinaceous infectious particles cause scrapieQ29547678
Prion and prejudice: normal protein and the synapseQ31874508
Cellular biology of prion diseasesQ33558398
Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP.Q33575860
Transmissible and genetic prion diseases share a common pathway of neurodegenerationQ33885223
Sensitive detection of pathological prion protein by cyclic amplification of protein misfoldingQ34084209
Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another proteinQ34294957
Role of microglia in neuronal cell death in prion diseaseQ47820922
Effects of oxidative stress on prion protein expression in PC12 cellsQ47924893
Prion protein is necessary for normal synaptic functionQ48087355
Prion protein peptide neurotoxicity can be mediated by astrocytesQ48127864
Prion proteins carrying pathogenic mutations are resistant to phospholipase cleavage of their glycolipid anchorsQ48171096
Influence of mutations associated with familial prion-related encephalopathies on biological activity of prion protein peptidesQ48231811
Astrocytic glutamate uptake and prion protein expressionQ48290049
Neurotoxicity of a prion protein fragmentQ48304223
Cellular prion protein localization in rodent and primate brainQ48388928
Prion protein fragment interacts with PrP-deficient cellsQ48467628
Toxicity of novel C-terminal prion protein fragments and peptides harbouring disease-related C-terminal mutationsQ48696484
Influence of amino acid substitutions related to inherited human prion diseases on the thermodynamic stability of the cellular prion proteinQ48769963
Familial mutations and the thermodynamic stability of the recombinant human prion proteinQ48784818
Truncated forms of the human prion protein in normal brain and in prion diseasesQ48807964
Identification of a protein that purifies with the scrapie prionQ48858653
Sublethal concentrations of prion peptide PrP106-126 or the amyloid beta peptide of Alzheimer's disease activates expression of proapoptotic markers in primary cortical neurons.Q48920441
Role of microglia and host prion protein in neurotoxicity of a prion protein fragmentQ49050987
Normal host prion protein necessary for scrapie-induced neurotoxicityQ49160879
Familial prion disease mutation alters the secondary structure of recombinant mouse prion protein: implications for the mechanism of prion formation.Q51986669
Prion peptide fragment PrP[106-126] forms distinct cation channel types.Q53904400
The role of prion peptide structure and aggregation in toxicity and membrane binding.Q54029824
Transmission of Two Subacute Spongiform Encephalopathies of Man (Kuru and Creutzfeldt–Jakob Disease) to New World MonkeysQ54526215
The cellular prion protein binds copper in vivoQ56058857
The Metabolism and Imaging in Live Cells of the Bovine Prion Protein in Its Native Form or Carrying Single Amino Acid SubstitutionsQ57356141
Altered circadian activity rhythms and sleep in mice devoid of prion proteinQ59071240
Proteasomal Degradation and N-terminal Protease Resistance of the Codon 145 Mutant Prion ProteinQ59505870
Mouse cortical cells lacking cellular PrP survive in culture with a neurotoxic PrP fragmentQ72568587
In vivo cytotoxicity of the prion protein fragment 106-126Q73010762
Cellular uptake of the prion protein fragment PrP106-126 in vitroQ73254939
PrP and β‐Amyloid Fragments Activate Different Neurotoxic Mechanisms in Cultured Mouse CellsQ73494469
The stimulation of inducible nitric-oxide synthase by the prion protein fragment 106--126 in human microglia is tumor necrosis factor-alpha-dependent and involves p38 mitogen-activated protein kinaseQ73794043
Effect of flupirtine on Bcl-2 and glutathione level in neuronal cells treated in vitro with the prion protein fragment (PrP106-126)Q73820551
Structural changes in a hydrophobic domain of the prion protein induced by hydration and by ala-->Val and pro-->Leu substitutionsQ74027634
Folding dynamics and energetics of recombinant prion proteinsQ34305029
The cellular prion protein binds copper in vivoQ34450742
A transmembrane form of the prion protein in neurodegenerative diseaseQ34454961
Normal prion protein has an activity like that of superoxide dismutase.Q34505606
Two-octapeptide repeat deletion of prion protein associated with rapidly progressive dementiaQ34516529
Prion infection impairs the cellular response to oxidative stressQ35581657
Separation and properties of cellular and scrapie prion proteinsQ35598678
Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58Q35921884
Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Sträussler-Scheinker diseaseQ36174780
Neurofibrillary tangles in Gerstmann-Sträussler-Scheinker syndrome with the A117V prion gene mutationQ36317012
Prion protein NMR structure and familial human spongiform encephalopathiesQ36317308
Normal and scrapie-associated forms of prion protein differ in their sensitivities to phospholipase and proteases in intact neuroblastoma cellsQ36802057
Location and properties of metal-binding sites on the human prion protein.Q37104327
Genetic influence on the structural variations of the abnormal prion proteinQ37422460
Two mutant prion proteins expressed in cultured cells acquire biochemical properties reminiscent of the scrapie isoform.Q37593764
A Mutant Prion Protein Displays an Aberrant Membrane Association When Expressed in Cultured CellsQ38290309
A monomer-dimer equilibrium of a cellular prion protein (PrPC) not observed with recombinant PrP.Q38308795
Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion diseaseQ39014762
Familial Creutzfeldt-Jakob disease. Codon 200 prion disease in Libyan JewsQ39241829
Prion protein with an E200K mutation displays properties similar to those of the cellular isoform PrP(C).Q39409269
Inherited prion diseases and transmission to rodentsQ40537021
Internalization of mammalian fluorescent cellular prion protein and N-terminal deletion mutants in living cellsQ40775514
Prion protein fragment PrP-(106-126) induces apoptosis via mitochondrial disruption in human neuronal SH-SY5Y cellsQ40782705
Mutant prion proteins are partially retained in the endoplasmic reticulumQ40783039
Most pathogenic mutations do not alter the membrane topology of the prion proteinQ40846248
Prion protein amyloidosisQ41061823
A synthetic peptide initiates Gerstmann-Sträussler-Scheinker (GSS) disease in transgenic miceQ41717349
Altered toxicity of the prion protein peptide PrP106-126 carrying the Ala(117)-->Val mutationQ41860073
A single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathyQ42688788
Prion proteins with different conformations accumulate in Gerstmann-Sträussler-Scheinker disease caused by A117V and F198S mutationsQ42746740
PrPSc-like prion protein peptide inhibits the function of cellular prion proteinQ42998109
Experimental transmission of Creutzfeldt-Jakob disease and related diseases to rodentsQ43213273
A 7-kDa prion protein (PrP) fragment, an integral component of the PrP region required for infectivity, is the major amyloid protein in Gerstmann-Sträussler-Scheinker disease A117V.Q43510340
Normal development and behaviour of mice lacking the neuronal cell-surface PrP proteinQ43522858
Ablation of the metal ion-induced endocytosis of the prion protein by disease-associated mutation of the octarepeat regionQ43645286
Aberrant metal binding by prion protein in human prion disease.Q43752347
Glycosylinositol phospholipid anchors of the scrapie and cellular prion proteins contain sialic acidQ45935884
A cytoskeletal mechanism for Ca2+ channel metabolic dependence and inactivation by intracellular Ca2+.Q46030546
Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchorQ46096263
Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C.Q46123074
Spontaneous neurodegeneration in transgenic mice with mutant prion proteinQ46249938
Prion protein selectively binds copper(II) ionsQ46492410
Scrapie prion protein contains a phosphatidylinositol glycolipidQ46814773
P433issue3
P921main subjectprion protein familyQ24724413
P304page(s)287-302
P577publication date2002-06-01
P1433published inMolecular NeurobiologyQ15716645
P1476titleMolecular advances in understanding inherited prion diseases
P478volume25

Reverse relations

cites work (P2860)
Q48557029Ancestral origins of the prion protein gene D178N mutation in the Basque Country
Q40645634Efficient and specific down-regulation of prion protein expression by RNAi
Q47695033Unexpected high testis-specific transcriptional activity of the cyclin T1 promoter in transgenic mice