β-Amyloid and the pathomechanisms of Alzheimer's disease: a comprehensive view

B Penke, F Bogár, L Fülöp - Molecules, 2017 - mdpi.com
Protein dyshomeostasis is the common mechanism of neurodegenerative diseases such as
Alzheimer's disease (AD). Aging is the key risk factor, as the capacity of the proteostasis …

New generation QuIC assays for prion seeding activity

CD Orru, JM Wilham, S Vascellari, AG Hughson… - Prion, 2012 - Taylor & Francis
The ability of abnormal TSE-associated forms of PrP to seed the formation of amyloid fibrils
from recombinant PrPSen has served as the basis for several relatively rapid and highly …

The real-time quaking-induced conversion assay for detection of human prion disease and study of other protein misfolding diseases

M Schmitz, M Cramm, F Llorens, D Müller-Cramm… - Nature protocols, 2016 - nature.com
The development and adaption of in vitro misfolded protein amplification systems has been
a major innovation in the detection of abnormally folded prion protein scrapie (PrPSc) in …

Protein misfolding cyclic amplification of infectious prions

R Morales, C Duran-Aniotz, R Diaz-Espinoza… - Nature protocols, 2012 - nature.com
Prions are proteinaceous infectious agents responsible for the transmission of prion
diseases. The lack of a procedure for cultivating prions in the laboratory has been a major …

Understanding the key features of the spontaneous formation of bona fide prions through a novel methodology that enables their swift and consistent generation

H Eraña, CM Díaz-Domínguez, JM Charco… - Acta neuropathologica …, 2023 - Springer
Among transmissible spongiform encephalopathies or prion diseases affecting humans,
sporadic forms such as sporadic Creutzfeldt–Jakob disease are the vast majority. Unlike …

Genesis of mammalian prions: from non-infectious amyloid fibrils to a transmissible prion disease

N Makarava, GG Kovacs, R Savtchenko… - PLoS …, 2011 - journals.plos.org
The transmissible agent of prion disease consists of a prion protein in its abnormal, β-sheet
rich state (PrPSc), which is capable of replicating itself according to the template-assisted …

Sialylation of Prion Protein Controls the Rate of Prion Amplification, the Cross-Species Barrier, the Ratio of PrPSc Glycoform and Prion Infectivity

E Katorcha, N Makarava, R Savtchenko… - PLoS …, 2014 - journals.plos.org
The central event underlying prion diseases involves conformational change of the cellular
form of the prion protein (PrPC) into the disease-associated, transmissible form (PrPSc) …

Evolution of diagnostic tests for chronic wasting disease, a naturally occurring prion disease of cervids

NJ Haley, JA Richt - Pathogens, 2017 - mdpi.com
Since chronic wasting disease (CWD) was first identified nearly 50 years ago in a captive
mule deer herd in the Rocky Mountains of the United States, it has slowly spread across …

Prions: beyond a single protein

AS Das, WQ Zou - Clinical microbiology reviews, 2016 - Am Soc Microbiol
Since the term protein was first coined in 1838 and protein was discovered to be the
essential component of fibrin and albumin, all cellular proteins were presumed to play …

Molecular pathology, classification, and diagnosis of sporadic human prion disease variants

P Parchi, D Saverioni - Folia neuropathologica, 2012 - termedia.pl
Human prion diseases are a unique group of transmissible neurodegenerative diseases that
occur as sporadic, familial or acquired disorders and show a wide range of phenotypic …