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) …

Prions: protein aggregation and infectious diseases

A Aguzzi, AM Calella - Physiological reviews, 2009 - journals.physiology.org
Transmissible spongiform encephalopathies (TSEs) are inevitably lethal neurodegenerative
diseases that affect humans and a large variety of animals. The infectious agent responsible …

Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis

G Mallucci, A Dickinson, J Linehan, PC Klohn… - Science, 2003 - science.org
The mechanisms involved in prion neurotoxicity are unclear, and therapies preventing
accumulation of PrPSc, the disease-associated form of prion protein (PrP), do not …

Mammalian prion biology: one century of evolving concepts

A Aguzzi, M Polymenidou - Cell, 2004 - cell.com
Prions have been responsible for an entire century of tragic episodes. Fifty years ago, kuru
decimated the population of Papua New Guinea. Then, iatrogenic transmission of prions …

Doxycycline in Creutzfeldt-Jakob disease: a phase 2, randomised, double-blind, placebo-controlled trial

S Haïk, G Marcon, A Mallet, M Tettamanti… - The Lancet …, 2014 - thelancet.com
Summary Background Creutzfeldt-Jakob disease (CJD) is a fatal, untreatable prion
encephalopathy. Previous studies showed that doxycycline is effective in in-vitro and in-vivo …

Lysosomotropic agents and cysteine protease inhibitors inhibit scrapie-associated prion protein accumulation

K Doh-Ura, T Iwaki, B Caughey - Journal of virology, 2000 - Am Soc Microbiol
We report that lysosomotropic agents and cysteine protease inhibitors inhibited protease-
resistant prion protein accumulation in scrapie-infected neuroblastoma cells. The inhibition …

Porphyrin and phthalocyanine antiscrapie compounds

SA Priola, A Raines, WS Caughey - Science, 2000 - science.org
The transmissible spongiform encephalopathies (TSEs) are fatal, neurodegenerative
diseases for which no effective treatments are available. The likelihood that a bovine form of …

[HTML][HTML] Anti-amyloidogenic activity of tetracyclines: studies in vitro

G Forloni, L Colombo, L Girola, F Tagliavini… - FEBS letters, 2001 - Elsevier
Cerebral deposition of β-amyloid is a major neuropathological feature in Alzheimer's
disease. Here we show that tetracyclines, tetracycline and doxycycline, classical antibiotics …

The systemic amyloidoses: clearer understanding of the molecular mechanisms offers hope for more effective therapies

G Merlini, P Westermark - Journal of internal medicine, 2004 - Wiley Online Library
Knowledge about the systemic amyloidoses has increased considerably during the last few
years. This group of diseases is characterized by great biochemical variability, including at …

Elimination of prions by branched polyamines and implications for therapeutics

S Supattapone, HOB Nguyen… - Proceedings of the …, 1999 - National Acad Sciences
We report that branched polyamines, including polyamidoamide dendimers,
polypropyleneimine, and polyethyleneimine, are able to purge PrPSc, the protease-resistant …