Prions, prionoids and protein misfolding disorders

C Scheckel, A Aguzzi - Nature Reviews Genetics, 2018 - nature.com
Prion diseases are progressive, incurable and fatal neurodegenerative conditions. The term
'prion'was first nominated to express the revolutionary concept that a protein could be …

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 …

Bmi-1 dependence distinguishes neural stem cell self-renewal from progenitor proliferation

AV Molofsky, R Pardal, T Iwashita, IK Park, MF Clarke… - Nature, 2003 - nature.com
Stem cells persist throughout life by self-renewing in numerous tissues including the central
and peripheral nervous systems. This raises the issue of whether there is a conserved …

T-cell-mediated cytotoxicity toward platelets in chronic idiopathic thrombocytopenic purpura

B Olsson, PO Andersson, M Jernås, S Jacobsson… - Nature medicine, 2003 - nature.com
Chronic idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder that is
characterized by increased platelet destruction and is believed to be autoantibody mediated …

The transcellular spread of cytosolic amyloids, prions, and prionoids

A Aguzzi, L Rajendran - Neuron, 2009 - cell.com
Recent reports indicate that a growing number of intracellular proteins are not only prone to
pathological aggregation but can also be released and" infect" neighboring cells. Therefore …

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 …

How does Parkinson's disease begin? Perspectives on neuroanatomical pathways, prions, and histology

P Borghammer - Movement Disorders, 2018 - Wiley Online Library
Parkinson's disease (PD) is a multisystem disorder with involvement of the peripheral
nervous system. Misfolding and aggregation of α‐synuclein is central to the pathogenesis of …

Molecular mechanisms of prion pathogenesis

A Aguzzi, C Sigurdson… - Annu. Rev. Pathol. Mech …, 2008 - annualreviews.org
Prion diseases are infectious neurodegenerative diseases occurring in humans and animals
with an invariably lethal outcome. One fundamental mechanistic event in prion diseases is …

The state of the prion

C Weissmann - Nature Reviews Microbiology, 2004 - nature.com
There is little doubt that the main component of the transmissible agent of spongiform
encephalopathies—the prion—is a conformational variant of the ubiquitous host protein …

Cellular and molecular mechanisms of prion disease

CJ Sigurdson, JC Bartz, M Glatzel - Annual Review of Pathology …, 2019 - annualreviews.org
Prion diseases are rapidly progressive, incurable neurodegenerative disorders caused by
misfolded, aggregated proteins known as prions, which are uniquely infectious. Remarkably …