Effect of the E200K mutation on prion protein metabolism. Comparative study of a cell model and human brain.
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Fatal prion disease in a mouse model of genetic E200K Creutzfeldt-Jakob diseaseProteasomes and ubiquitin are involved in the turnover of the wild-type prion proteinCellular phenotyping of secretory and nuclear prion proteins associated with inherited prion diseasesSelective processing and metabolism of disease-causing mutant prion proteins.Refinement of under-determined loops of Human Prion Protein by database-derived distance constraintsCharacteristic CSF prion seeding efficiency in humans with prion diseasesPrion protein biosynthesis and its emerging role in neurodegeneration.Role of prion protein aggregation in neurotoxicity.Mutations in the amino terminus of foamy virus Gag disrupt morphology and infectivity but do not target assembly.H-type bovine spongiform encephalopathy: complex molecular features and similarities with human prion diseasesCharacterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease.Coexistence of two forms of disease-associated prion protein in extracerebral tissues of cattle infected with H-type bovine spongiform encephalopathy.The consequences of pathogenic mutations to the human prion protein.Selective vulnerability to neurodegenerative disease: the curious case of Prion Protein.Genetic Creutzfeldt-Jakob disease and fatal familial insomnia: insights into phenotypic variability and disease pathogenesis.Phenotypic variability of sporadic human prion disease and its molecular basis: past, present, and future.Proteomic profiling of cerebrospinal fluid in Creutzfeldt-Jakob disease.Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins.Mutant prion protein D202N associated with familial prion disease is retained in the endoplasmic reticulum and forms 'curly' intracellular aggregatesExosomal cellular prion protein drives fibrillization of amyloid beta and counteracts amyloid beta-mediated neurotoxicity.Hypoxia induces expression of a GPI-anchorless splice variant of the prion protein.A Copine family member, Cpne8, is a candidate quantitative trait gene for prion disease incubation time in mouse.Mutant prion proteins are partially retained in the endoplasmic reticulum.Codon 129 polymorphism and the E200K mutation do not affect the cellular prion protein isoform composition in the cerebrospinal fluid from patients with Creutzfeldt-Jakob disease.N-terminal truncation of prion protein affects both formation and conformation of abnormal protease-resistant prion protein generated in vitro.Biochemical fingerprints of prion diseases: scrapie prion protein in human prion diseases that share prion genotype and type.Identification of distinct N-terminal truncated forms of prion protein in different Creutzfeldt-Jakob disease subtypes.Applications of the real-time quaking-induced conversion assay in diagnosis, prion strain-typing, drug pre-screening and other amyloidopathies.Flexible N-terminal region of prion protein influences conformation of protease-resistant prion protein isoforms associated with cross-species scrapie infection in vivo and in vitro.Heterogeneity of normal prion protein in two- dimensional immunoblot: presence of various glycosylated and truncated forms.A cluster of familial Creutzfeldt-Jakob disease mutations recapitulate conserved residues in Doppel: a case of molecular mimicry?
P2860
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P2860
Effect of the E200K mutation on prion protein metabolism. Comparative study of a cell model and human brain.
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
Effect of the E200K mutation o ...... a cell model and human brain.
@ast
Effect of the E200K mutation o ...... a cell model and human brain.
@en
type
label
Effect of the E200K mutation o ...... a cell model and human brain.
@ast
Effect of the E200K mutation o ...... a cell model and human brain.
@en
prefLabel
Effect of the E200K mutation o ...... a cell model and human brain.
@ast
Effect of the E200K mutation o ...... a cell model and human brain.
@en
P2093
P2860
P1476
Effect of the E200K mutation o ...... a cell model and human brain.
@en
P2093
P Gambetti
R B Petersen
S Capellari
P2860
P304
P356
10.1016/S0002-9440(10)64572-5
P407
P577
2000-08-01T00:00:00Z