High-resolution structure and strain comparison of infectious mammalian prions

A Kraus, F Hoyt, CL Schwartz, B Hansen, E Artikis… - Molecular cell, 2021 - cell.com
Within the extensive range of self-propagating pathologic protein aggregates of mammals,
prions are the most clearly infectious (eg,∼ 10 9 lethal doses per milligram). The structures …

Structural biology of ex vivo mammalian prions

E Artikis, A Kraus, B Caughey - Journal of Biological Chemistry, 2022 - ASBMB
The structures of prion protein (PrP)–based mammalian prions have long been elusive.
However, cryo-EM has begun to reveal the near-atomic resolution structures of fully …

Pathogenic prion structures at high resolution

B Caughey, HG Standke, E Artikis, F Hoyt… - PLoS …, 2022 - journals.plos.org
Numerous proteins are known to form ordered, self-propagating aggregates as underlying
causes of neurodegeneration in proteinopathies such as Alzheimer's, Parkinson's, and prion …

Parallel in-register intermolecular β-sheet architectures for prion-seeded prion protein (PrP) amyloids

BR Groveman, MA Dolan, LM Taubner, A Kraus… - Journal of Biological …, 2014 - ASBMB
Structures of the infectious form of prion protein (eg PrP Sc or PrP-Scrapie) remain poorly
defined. The prevalent structural models of PrP Sc retain most of the native α-helices of the …

The structure of the infectious prion protein: experimental data and molecular models

JR Requena, H Wille - Prion, 2014 - Taylor & Francis
The structures of the infectious prion protein, PrPSc, and that of its proteolytically truncated
variant, PrP 27–30, have evaded experimental determination due to their insolubility and …

Structure of an infectious mammalian prion

A Kraus, F Hoyt, CL Schwartz, B Hansen, AG Hughson… - BioRxiv, 2021 - biorxiv.org
Classical mammalian prions are assemblies of prion protein molecules that are
extraordinarily transmissible, with a microgram of protein containing up to 108 lethal doses …

Prions and the potential transmissibility of protein misfolding diseases

A Kraus, BR Groveman… - Annual review of …, 2013 - annualreviews.org
Prions, or infectious proteins, represent a major frontier in the study of infectious agents. The
prions responsible for mammalian transmissible spongiform encephalopathies (TSEs) are …

Toward the molecular basis of inherited prion diseases: NMR structure of the human prion protein with V210I mutation

I Biljan, G Ilc, G Giachin, A Raspadori, I Zhukov… - Journal of molecular …, 2011 - Elsevier
The development of transmissible spongiform encephalopathies (TSEs) is associated with
the conversion of the cellular prion protein (PrPC) into a misfolded, pathogenic isoform …

[HTML][HTML] Heparin binding confers prion stability and impairs its aggregation

TCRG Vieira, Y Cordeiro, B Caughey… - The FASEB Journal, 2014 - ncbi.nlm.nih.gov
The conversion of the prion protein (PrP) into scrapie PrP (PrP Sc) is a central event in prion
diseases. Several molecules work as cofactors in the conversion process, including …

Common structural traits across pathogenic mutants of the human prion protein and their implications for familial prion diseases

G Rossetti, X Cong, R Caliandro, G Legname… - Journal of molecular …, 2011 - Elsevier
Human (Hu) familial prion diseases are associated with about 40 point mutations of the
gene coding for the prion protein (PrP). Most of the variants associated with these mutations …