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Prions (2):
Recent article
Prions
Abstract
Prion replication in the periphery precedes neuroinvasion in many experimental rodent scrapie models, and in natural sheep scrapie and chronic wasting disease (CWD) in cervids. Prions propagate in the germinal centers of secondary lymphoid organs and are strongly associated with foll...
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PMID: 22043911
PDF is available here.
PDF is available here.
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Abstract
We combine the accelerated sampling properties of replica exchange molecular dynamics simulations based on the OPEP coarse-grained potential with the atomic resolution description of interactions provided by all-atom MD simulations, and investigate the oligomerization process of the GNNQQNY for thre...
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PMID: 21625573
PDF is available here.
PDF is available here.
Abstract
We determined the extent of caspase activation in the brain and peripheral organs of mice that showed clinical signs after intracerebral inoculation with mouse-adapted prions by in vivo administration of a red fluorescent pan-caspase inhibitor, sulforhodamine B-Val-Ala-Asp(OMe)-fluoromethylketone. F...
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PMID: 21343883
PDF is available here.
PDF is available here.
Abstract
We here studied the globular domain of this mutant protein by molecular dynamics simulations. Compared to the wide-type protein, the mutant has similar dynamics and stability profiles in our simulation. Conformational rearrangements are concentrated around the mutation site, due to the introduction...
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PMID: 20919751
PDF is available here.
PDF is available here.
Abstract
We report the clinical, neuropsychologic, imaging, genetic, and neuropathologic features of a patient with familial Creutzfeldt-Jakob disease, associated with a very rare PRNP mutation at T188R. The patient presented with prominent behavioral changes in addition to the more typical cognitive and mot...
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PMID: 21107135
PDF is available here.
PDF is available here.
Abstract
Prions are an unusual form of epigenetics: Their stable inheritance and complex phenotypes come about through protein folding rather than nucleic acid-associated changes. With intimate ties to protein homeostasis and a remarkable sensitivity to stress, prions are a robust mechanism that links enviro...
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PMID: 21030648
PDF is available here.
PDF is available here.
Abstract
We found that protein conformation determined the size distribution of aggregates through its interactions with a molecular chaperone. Shifts in this range created variations in aggregate abundance among cells because of a size threshold for transmission, and this heterogeneity, along with aggregate...
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PMID: 21030659
PDF is available here.
PDF is available here.
Journal of Biological Chemistry (285)42 2010
Abstract
We studied a range of synthetic peptides: specifically those equating to the N1 (residues 23-110) and N2 (23-89) fragments derived from constitutive processing of PrP(C) and including those representing arbitrarily defined component domains of the N terminus of mouse prion protein. Utilizing more ph...
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PMID: 20679345
PDF is available here.
PDF is available here.
Journal of Biological Chemistry (285)42 2010
Abstract
We utilize amide hydrogen exchange measurements to probe the organization of the peptide in its fibrillar form. We determined the extent of hydrogen exchange first by tandem proteolysis, liquid chromatography, and mass spectrometry (HXMS) and then by exchange-quenched NMR. Although single amide reso...
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PMID: 20679344
PDF is available here.
PDF is available here.
Oligonucleotides (20)5 2010
Abstract
Biochemical Genetics (48)9-10 2010
Abstract
Six polymorphic sites of the bovine prion protein gene (PRNP) were genotyped in 569 animals of Asian native cattle, Japanese breeds, purebred mythun (Bos frontalis), and mythun x cattle composite animals. At the 23-bp indel site, a deletion (23-) allele was a major allele in all populations except m...
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PMID: 20623331
PDF is available here.
PDF is available here.
Abstract
We hypothesize suppression of the PRNP gene expression could raise resistance to BSE in cattle by using vector-based small interfering RNA (siRNA) expression systems. Therefore, the objective was to screen effective DNA-encoding short hairpin RNAs (shRNAs) which could knockdown the PRNP gene express...
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PMID: 19821149
PDF is available here.
PDF is available here.
Abstract
Prions are pathogenic proteins and are the cause for spongiform encephalopathies. Pathogenic prions differ from physiologically common non-pathogenic prions only in their sterical structure. Upon infection by a pathogenic prion protein, a series of reactions is initiated in which common non-pathogen...
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PMID: 21192443
PDF is available here.
PDF is available here.
Abstract
No pathogenic prions were found in any of the 1142 tested specimens. One specimen revealed weak positivity at initial screening; however, repeated examination of the specimen and other specimens from different locations in the brain of the same donor did not confirm the presence of pathogenic prions...
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PMID: 20520532
PDF is available here.
PDF is available here.
Abstract
Our report on the first Dutch patient with PSPr further expands the spectrum of prionopathies and exemplifies the need to re-evaluate cases of atypical prion disease....
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PMID: 20547632
PDF is available here.
PDF is available here.
Biophysical Journal (99)4 2010
Abstract
We previously generated a toxic mutant of the nontoxic HET-s((218-289)) amyloid in yeast. Here we report that toxic and nontoxic amyloids differ not only in their structures but also in their assembling process. We used multiple and complementary methods to investigate the intermediates formed by th...
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PMID: 20713008
PDF is available here.
PDF is available here.
Abstract
We here demonstrate paramagnetic relaxation-enhanced (PRE) NMR spectroscopy to be a powerful tool unraveling structural information about epitope-orientation in a groove spanned by the complementary determining regions. In particular, we utilize the spin label TOAC, which is fused to the peptidic ep...
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PMID: 20476792
PDF is available here.
PDF is available here.
Vox Sanguinis (99)2 2010
Abstract
Variant Creutzfeldt-Jakob (vCJD) is a fatal transfusion transmissible prion infection. No test for vCJD in the donor population is currently available. Therefore, prion removal by filtration of red cell concentrate (RCC) is an attractive option for prevention.
Twenty patients were recruited with eth...
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PMID: 20345513
PDF is available here.
PDF is available here.
Journal of General Virology (91)Pt 8 2010
Abstract
We have generated PrP-specific antibodies (known as PrioV) by immunization of camels with murine scrapie material adsorbed to immunomagnetic beads. The PrioV antibodies display a range of specificities with some recognizing the PrP(27-30) proteinase K-resistant fragment, others specific for PrP(C) a...
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PMID: 20375226
PDF is available here.
PDF is available here.
Journal of General Virology (91)Pt 8 2010
Abstract
Twenty-four atypical scrapie cases from sheep with different prion protein genotypes from Great Britain were transmitted to transgenic tg338 and/or TgshpXI mice expressing sheep PrP alleles, but failed to transmit to wild-type mice. Mean incubation periods were 200-300 days in tg338 mice and 300-500...
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PMID: 20392900
PDF is available here.
PDF is available here.
Journal of Virology (84)16 2010
Abstract
We describe a modification of the scrapie cell assay allowing evaluation of prions causing chronic wasting disease, a naturally occurring transmissible spongiform encephalopathy. We compare this cervid prion cell assay to bioassays in transgenic mice, the only other existing method for quantificatio...
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PMID: 20519392
PDF is available here.
PDF is available here.
Abstract
We report a 75-yr-old woman with familial CJD carrying a V180I mutation which features late onset, slow progression, no periodic sharp wave complexes on electroencephalography, and extensive cortical ribboning with spared the cerebellum and the medial occipital lobes posterior to the parieto-occipit...
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PMID: 20592908
PDF is available here.
PDF is available here.
Abstract
We recently reported the intraspecies transmission of L-type atypical bovine spongiform encephalopathy (BSE). To clarify the peripheral pathogenesis of L-type BSE, we studied prion distribution in nerve and lymphoid tissues obtained from experimentally challenged cattle. As with classical BSE prions...
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PMID: 20587193
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
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PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
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PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
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PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
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PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
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PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
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PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
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PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
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PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
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PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Human Mutation (31)7 2010
Abstract
We present novel allele combinations, healthy control population data, results of screening the PRNP ORF in DNA from the entire referral series and the CEPH human genome diversity cell line panel. Of the 10 alleles detected in patients for which detailed cases histories are presented, 4 are unreport...
|
PMID: 20583301
PDF is available here.
PDF is available here.
Veterinary Record (167)1 2010
Abstract
Abstract
We confirm this link by finding that deletion of STI1 both suppresses Ssa1-21 impairment of [PSI(+)] and blocks Hsp104 curing of [PSI(+)]. Hsp104's tetratricopeptide repeat (TPR) interaction motif was dispensable for curing; however, cells expressing Sti1 defective in Hsp70 or Hsp90 interaction cure...
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PMID: 20479121
PDF is available here.
PDF is available here.
Journal of Neuroscience (30)27 2010
Abstract
We identify a novel role for contactin-associated protein (Caspr) as an inhibitory cue that reduces neurite outgrowth from CNS neurons. We show that proteolysis of Caspr at the cell surface is regulated by the cellular form of prion protein (PrP), which directly binds to Caspr. PrP inhibits Reelin-m...
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PMID: 20610764
PDF is available here.
PDF is available here.
Molecular Cell (38)6 2010
Abstract
We show that HET-S fails to form fibrils in vitro and that it inhibits HET-s PFD fibrillization in trans. In vivo analyses indicate that beta-structuring of the HET-S PFD is required for HET-S activity. The crystal structures of the globular domains of HET-s and HET-S are highly similar, comprising...
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PMID: 20620958
PDF is available here.
PDF is available here.
Abstract
We prove that the global transcriptional regulator Sfp1 can become a prion corresponding to the prion-like determinant [ISP(+)] described earlier. We show that SFP1 deletion causes an irreversible [ISP(+)] loss, whereas increased SFP1 expression induces [ISP(+)] appearance. Cells that display the [I...
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PMID: 20498075
PDF is available here.
PDF is available here.
Abstract
We demonstrate that the yeast prion protein Sup35 can access an infectious conformation in Escherichia coli cells and that formation of this material is greatly stimulated by the presence of a transplanted [PSI(+)] inducibility factor, a distinct prion that is required for Sup35 to undergo spontaneo...
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PMID: 20484678
PDF is available here.
PDF is available here.
Abstract
We hypothesized that binding to soil enhances prion resistance to enzymatic digestion, thereby facilitating prion longevity in the environment and providing protection from host degradation. We characterized the performance of a commercially available subtilisin enzyme, Prionzyme, to degrade soil-bo...
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PMID: 20450190
PDF is available here.
PDF is available here.
Biophysical Chemistry (149)1-2 2010
Abstract
We present the results of MD simulations that provide the atomistic details of the process, by which the small molecules may destabilize the ordered amyloid oligomers formed by the model hexapeptide. We select a heptapeptide fragment (GNNQQNY) from Sup-35 yeast prion protein, which is capable to for...
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PMID: 20456856
PDF is available here.
PDF is available here.
Journal of General Virology (91)Pt 6 2010
Abstract
These results demonstrate that CWD can be transmitted by aerosol (as well as nasal) exposure and suggest that exposure via the respiratory system merits consideration for prion disease transmission and biosafety....
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PMID: 20164261
PDF is available here.
PDF is available here.
Rinsho Shinkeigaku (50)5 2010
Abstract
Prion disease develops when normal prion proteins change into transmissible abnormal prion proteins and the converted proteins accumulate in the brain. The Japanese Creutzfeldt-Jakob Disease (CJD) Surveillance Committee has identified 1320 patients with prion diseases in the 10 years since 1999 (cla...
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PMID: 20535976
PDF is available here.
PDF is available here.
Abstract
Binding of BF-227 to PrP plaques was confirmed using brain samples from autopsy-confirmed GSS cases. In clinical PET study, significantly higher retention of BF-227 was detected in the cerebellum, thalamus and lateral temporal cortex of GSS patients compared to that in the corresponding tissues of n...
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PMID: 20016895
PDF is available here.
PDF is available here.
Abstract
The prion protein (PrP) is implicitly involved in the pathogenesis of transmissible spongiform encephalopathies (TSEs). The conversion of normal cellular PrP (PrP(C)), a protein that is predominantly alpha-helical, to a beta-sheet-rich isoform (PrP(Sc)), which has a propensity to aggregate, is the k...
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PMID: 20206551
PDF is available here.
PDF is available here.
Veterinary Pathology (47)3 2010
Abstract
Eyes and nuclei of the visual pathways in the brain were examined in 30 Rocky Mountain elk (Cervus elaphus nelsoni) representing 3 genotypes of the prion protein gene PRNP (codon 132: MM, ML, or LL). Tissues were examined for the presence of the abnormal isoform of the prion protein associated with...
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PMID: 20382822
PDF is available here.
PDF is available here.
Biophysical Journal (98)8 2010
Abstract
Prion diseases are neurodegenerative disorders of the central nervous system that are associated with the misfolding of the prion protein (PrP). PrP is glycosylphosphatidylinositol-anchored, and therefore the hydrophobic membrane environment may influence the process of prion conversion. This study...
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PMID: 20409471
PDF is available here.
PDF is available here.