Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation.
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Pseudoknots in prion protein mRNAs confirmed by comparative sequence analysis and pattern searchingExpression of mutant or cytosolic PrP in transgenic mice and cells is not associated with endoplasmic reticulum stress or proteasome dysfunctionMutant PrP suppresses glutamatergic neurotransmission in cerebellar granule neurons by impairing membrane delivery of VGCC α(2)δ-1 SubunitEarly onset prion disease from octarepeat expansion correlates with copper binding propertiesDe novo generation of a transmissible spongiform encephalopathy by mouse transgenesisPrion protein insertional mutations increase aggregation propensity but not fiber stability.Molecular distinction between pathogenic and infectious properties of the prion protein.Immunopurification of pathological prion protein aggregatesBax deletion prevents neuronal loss but not neurological symptoms in a transgenic model of inherited prion disease.A protease-resistant 61-residue prion peptide causes neurodegeneration in transgenic mice.Huntington disease phenocopy is a familial prion disease.Infectious particles, stress, and induced prion amyloids: a unifying perspective.What is the basis of transmissible spongiform encephalopathy induced neurodegeneration and can it be repaired?High titers of transmissible spongiform encephalopathy infectivity associated with extremely low levels of PrPSc in vivo.Prion protein misfolding.Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.Prion diseases: from molecular biology to intervention strategies.Prion protein repeat expansion results in increased aggregation and reveals phenotypic variability.Stressing out the ER: a role of the unfolded protein response in prion-related disordersOctapeptide repeat insertions increase the rate of protease-resistant prion protein formationThe Immp2l mutation causes age-dependent degeneration of cerebellar granule neurons prevented by antioxidant treatment.Prion neurotoxicity: insights from prion protein mutantsGFP-tagged mutant prion protein forms intra-axonal aggregates in transgenic miceMutant PrPSc conformers induced by a synthetic peptide and several prion strainsAggregated, wild-type prion protein causes neurological dysfunction and synaptic abnormalities.PrP aggregation can be seeded by pre-formed recombinant PrP amyloid fibrils without the replication of infectious prionsPrion protein with an insertional mutation accumulates on axonal and dendritic plasmalemma and is associated with distinctive ultrastructural changes.Spontaneous generation of infectious prion disease in transgenic mice.Synaptic dysfunction in prion diseases: a trafficking problem?A computationally designed DNA aptamer template with specific binding to phosphatidylserine.Analysis of the hippocampal proteome in ME7 prion disease reveals a predominant astrocytic signature and highlights the brain-restricted production of clusterin in chronic neurodegeneration.De novo mammalian prion synthesis.Immunotherapy for prion diseases: opportunities and obstacles.Myelin damage due to local quantitative abnormalities in normal prion levels: evidence from subacute combined degeneration and multiple sclerosis.Mechanisms of prion-induced neurodegeneration.Misfolding leads the way to unraveling signaling pathways in the pathophysiology of prion diseases.Experimental Models of Inherited PrP Prion Diseases.Insights into Mechanisms of Transmission and Pathogenesis from Transgenic Mouse Models of Prion Diseases.Overstimulation of PrPC signaling pathways by prion peptide 106-126 causes oxidative injury of bioaminergic neuronal cells.Genetic mapping of activity determinants within cellular prion proteins: N-terminal modules in PrPC offset pro-apoptotic activity of the Doppel helix B/B' region.
P2860
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P2860
Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation.
description
2000 nî lūn-bûn
@nan
2000年の論文
@ja
2000年論文
@yue
2000年論文
@zh-hant
2000年論文
@zh-hk
2000年論文
@zh-mo
2000年論文
@zh-tw
2000年论文
@wuu
2000年论文
@zh
2000年论文
@zh-cn
name
Accumulation of protease-resis ...... ng a PrP insertional mutation.
@en
type
label
Accumulation of protease-resis ...... ng a PrP insertional mutation.
@en
prefLabel
Accumulation of protease-resis ...... ng a PrP insertional mutation.
@en
P2093
P2860
P356
P1476
Accumulation of protease-resis ...... ng a PrP insertional mutation.
@en
P2093
B Drisaldi
D A Harris
P Piccardo
P2860
P304
P356
10.1073/PNAS.97.10.5574
P407
P577
2000-05-01T00:00:00Z