Models of amyloid seeding in Alzheimer's disease and scrapie: mechanistic truths and physiological consequences of the time-dependent solubility of amyloid …

JD Harper, PT Lansbury Jr - Annual review of biochemistry, 1997 - annualreviews.org
Ordered protein aggregation in the brain is a hallmark of Alzheimer's disease and scrapie.
The disease-specific amyloid fibrils comprise primarily a single protein, amyloid β, in …

Prion diseases of humans and animals: their causes and molecular basis

J Collinge - Annual review of neuroscience, 2001 - annualreviews.org
▪ Abstract Prion diseases are transmissible neurodegenerative conditions that include
Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) …

Direct observation of the interconversion of normal and toxic forms of α-synuclein

N Cremades, SIA Cohen, E Deas, AY Abramov… - Cell, 2012 - cell.com
Here, we use single-molecule techniques to study the aggregation of α-synuclein, the
protein whose misfolding and deposition is associated with Parkinson's disease. We identify …

Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding

GP Saborio, B Permanne, C Soto - Nature, 2001 - nature.com
Prions are the infectious agents responsible for transmissible spongiform encephalopathies.
The principal component of prions is the glycoprotein PrPSc, which is a conformationally …

Quenching quorum-sensing-dependent bacterial infection by an N-acyl homoserine lactonase

YH Dong, LH Wang, JL Xu, HB Zhang, XF Zhang… - Nature, 2001 - nature.com
Bacterial cells sense their population density through a sophisticated cell–cell
communication system and trigger expression of particular genes when the density reaches …

Ultrastructural and biochemical classification of pathogenic tau, α-synuclein and TDP-43

A Tarutani, T Adachi, H Akatsu, Y Hashizume… - Acta …, 2022 - Springer
Intracellular accumulation of abnormal proteins with conformational changes is the defining
neuropathological feature of neurodegenerative diseases. The pathogenic proteins that …

The acid-mediated denaturation pathway of transthyretin yields a conformational intermediate that can self-assemble into amyloid

Z Lai, W Colón, JW Kelly - Biochemistry, 1996 - ACS Publications
Transthyretin (TTR) amyloid fibril formation is observed during partial acid denaturation and
while refolding acid-denatured TTR, implying that amyloid fibril formation results from the …

Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein

GC Telling, M Scott, J Mastrianni, R Gabizon, M Torchia… - Cell, 1995 - cell.com
Transgenic (Tg) mice expressing human (Hu) and chimerit prion protein (PrP) genes were
inoculated with brain extracts from humans with inherited or sporadic prion disease to …

Formation of native prions from minimal components in vitro

NR Deleault, BT Harris, JR Rees… - Proceedings of the …, 2007 - pnas.org
The conformational change of a host protein, PrPC, into a disease-associated isoform,
PrPSc, appears to play a critical role in the pathogenesis of prion diseases such as …

Protein aggregation in crowded environments

RJ Ellis, AP Minton - 2006 - degruyter.com
The generic tendency of proteins to aggregate into non-functional, and sometimes cytotoxic,
structures poses a universal problem for all types of cell. This tendency is greatly …