Prion-Like Proteins in Phase Separation and Their Link to Disease
Aberrant protein folding underpins many neurodegenerative diseases as well as certain myopathies and cancers. Protein misfolding can be driven by the presence of distinctive prion and prion-like regions within certain proteins. These prion and prion-like
Macy L. Sprunger, Meredith E. Jackrel
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Anti-prion drug mPPIg5 inhibits PrP(C) conversion to PrP(Sc). [PDF]
Prion diseases, also known as transmissible spongiform encephalopathies, are a group of fatal neurodegenerative diseases that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans.
Jeremy C. Simpson (29225) +31 more
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Sequence features governing aggregation or degradation of prion-like proteins. [PDF]
Enhanced protein aggregation and/or impaired clearance of aggregates can lead to neurodegenerative disorders such as Alzheimer's Disease, Huntington's Disease, and prion diseases.
Sean M Cascarina +3 more
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Prion Diseases: A Unique Transmissible Agent or a Model for Neurodegenerative Diseases?
The accumulation and propagation in the brain of misfolded proteins is a pathological hallmark shared by many neurodegenerative diseases such as Alzheimer’s disease (Aβ and tau), Parkinson’s disease (α-synuclein), and prion disease (prion protein ...
Diane L. Ritchie, Marcelo A. Barria
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Pros and cons of a prion-like pathogenesis in Parkinson's disease [PDF]
Background: Parkinson's disease (PD) is a slowly progressive neurodegenerative disorder which affects widespread areas of the brainstem, basal ganglia and cerebral cortex.
Chapman Joab +10 more
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Raman optical activity demonstrates poly(L-proline) II helix in the N-terminal region of the ovine prion protein: Implications for function and misfunction [PDF]
The aqueous solution structure of the full-length recombinant ovine prion protein PrP25-233, together with that of the N-terminal truncated version PrP94-233, have been studied using vibrational Raman optical activity (ROA) and ultraviolet circular ...
Rhie, A G O +18 more
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Editorial: Prion and prion-like proteins in neurodegenerative diseases
Mattia Toni, Simona Eleuteri
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Prion protein self-peptides modulate prion interactions and conversion [PDF]
Background: Molecular mechanisms underlying prion agent replication, converting host-encoded cellular prion protein (PrPC) into the scrapie associated isoform (PrPSc), are poorly understood.
Bossers, A. +12 more
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Prion-Like Domains in Phagobiota
Prions are molecules characterized by self-propagation, which can undergo a conformational switch leading to the creation of new prions. Prion proteins have originally been associated with the development of mammalian pathologies; however, recently they ...
George Tetz, Victor Tetz
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Evolution of sequence traits of prion-like proteins linked to amyotrophic lateral sclerosis (ALS) [PDF]
Prions are proteinaceous particles that can propagate an alternative conformation to further copies of the same protein. They have been described in mammals, fungi, bacteria and archaea.
Jiayi Luo, Paul M. Harrison
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