Accumulation of prion protein in the vagus nerve in creutzfeldt–jakob disease
Sign Up to like & getrecommendations! Published in 2019 at "Annals of Neurology"
DOI: 10.1002/ana.25451
Abstract: Disease‐associated proteins are thought to propagate along neuronal processes in neurodegenerative diseases. To detect disease‐associated prion protein (PrPSc) in the vagus nerve in different forms and molecular subtypes of Creutzfeldt–Jakob disease (CJD), we applied 3… read more here.
Keywords: cjd; disease; jakob disease; vagus nerve ... See more keywords
Structural Mechanism of Barriers to Interspecies Seeding Transmissibility of Full‐Length Prion Protein Amyloid
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DOI: 10.1002/cbic.201900218
Abstract: A puzzling feature of prion diseases is the cross‐species barriers. The detailed molecular mechanisms underlying these interspecies barriers remain poorly understood because of a lack of high‐resolution structural information on the scrapie isoform of the… read more here.
Keywords: 165 167; structural mechanism; prion; prion protein ... See more keywords
A Small‐Molecule Inhibitor of Prion Replication and Mutant Prion Protein Toxicity
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DOI: 10.1002/cmdc.201700302
Abstract: Into the fold: Prion diseases are neurodegenerative disorders characterized by the accumulation in the brain of a self-replicating, misfolded isoform (PrPSc ) of the cellular prion protein (PrPC ). No therapies are available for these… read more here.
Keywords: prion protein; prion; prion replication; toxicity ... See more keywords
The manifold role of octapeptide repeats in prion protein assembly
Sign Up to like & getrecommendations! Published in 2023 at "Peptide Science"
DOI: 10.1002/pep2.24303
Abstract: Prion protein misfolding is associated with fatal neurodegenerative disorders such as kuru, Creutzfeldt–Jakob disease, and several animal encephalopathies. While the C‐terminal 106–126 peptide has been well studied for its role in prion replication and toxicity,… read more here.
Keywords: prion protein; manifold role; role octapeptide; role ... See more keywords
Semi‐synthesis of murine prion protein by native chemical ligation and chemical activation for preparation of polypeptide‐α‐thioester
Sign Up to like & getrecommendations! Published in 2017 at "Journal of Peptide Science"
DOI: 10.1002/psc.3008
Abstract: Prions are suspected as pathogen of the fatal transmissible spongiform encephalopathies. Strategies to access homogenous prion protein (PrP) are required to fully comprehend the molecular mechanism of prion diseases. However, the polypeptide fragments from PrP… read more here.
Keywords: prion protein; chemical ligation; chemical; synthesis ... See more keywords
The function of the cellular prion protein in health and disease
Sign Up to like & getrecommendations! Published in 2017 at "Acta Neuropathologica"
DOI: 10.1007/s00401-017-1790-y
Abstract: The essential role of the cellular prion protein (PrPC) in prion disorders such as Creutzfeldt–Jakob disease is well documented. Moreover, evidence is accumulating that PrPC may act as a receptor for protein aggregates and transduce… read more here.
Keywords: protein; prpc; prion; disease ... See more keywords
Transmissible amyloid protein: evidence from iatrogenic CJD
Sign Up to like & getrecommendations! Published in 2018 at "Journal of Neurology"
DOI: 10.1007/s00415-018-8927-3
Abstract: Creutzfeldt–Jakob disease (CJD) is a progressive neurodegenerative condition caused by the accumulation of toxic forms of prion protein in the brain. In 1985, reports emerged of patients who had developed CJD following treatment with cadaveric… read more here.
Keywords: protein; evidence; pathology; iatrogenic cjd ... See more keywords
Ultra-efficient Amplification of Abnormal Prion Protein by Modified Protein Misfolding Cyclic Amplification with Electric Current
Sign Up to like & getrecommendations! Published in 2017 at "Molecular Neurobiology"
DOI: 10.1007/s12035-017-0431-8
Abstract: Prion diseases are clinically diagnosed and confirmed upon post-mortem histopathological examination of brain tissue. The only reliable molecular marker for prion diseases is abnormal prion protein (PrPSc), a pathologically conformed prion protein that primarily accumulates… read more here.
Keywords: prion; prion protein; prion diseases; abnormal prion ... See more keywords
Epigenetic Control of the Notch and Eph Signaling Pathways by the Prion Protein: Implications for Prion Diseases
Sign Up to like & getrecommendations! Published in 2019 at "Molecular Neurobiology"
DOI: 10.1007/s12035-018-1193-7
Abstract: Among the ever-growing number of self-replicating proteins involved in neurodegenerative diseases, the prion protein PrP remains the most infamous for its central role in transmissible spongiform encephalopathies (TSEs). In these diseases, pathogenic prions propagate through… read more here.
Keywords: prpc; prion; notch eph; eph signaling ... See more keywords
13C and 15N chemical shift assignments of A117V and M129V human Y145Stop prion protein amyloid fibrils.
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DOI: 10.1007/s12104-020-09981-4
Abstract: The C-terminally truncated Y145Stop variant of prion protein (PrP23-144) has been linked to a heritable prionopathy in humans and is also capable of triggering a transmissible prion disease in mice. PrP23-144 can be converted from… read more here.
Keywords: prion protein; a117v m129v; chemical shift;
1H, 13C, 15N backbone and side-chain resonance assignments of the pathogenic G131V mutant of human prion protein (91-231).
Sign Up to like & getrecommendations! Published in 2021 at "Biomolecular NMR assignments"
DOI: 10.1007/s12104-021-10022-x
Abstract: Human prion disease, also known as transmissible spongiform encephalopathy (TSEs), is caused by the conformational conversion of the normal cellular prion protein (PrPC) into the scrapie form (PrPSc). Pathogenic point mutations of prion proteins typically… read more here.
Keywords: prion; human prion; prion protein; backbone ... See more keywords