Physiology of the prion protein

R Linden, VR Martins, MAM Prado… - Physiological …, 2008 - journals.physiology.org
Prion diseases are transmissible spongiform encephalopathies (TSEs), attributed to
conformational conversion of the cellular prion protein (PrPC) into an abnormal conformer …

Prions and their partners in crime

B Caughey, GS Baron - Nature, 2006 - nature.com
Prions, the infectious agents of transmissible spongiform encephalopathies (TSEs), have
defied full characterization for decades. The dogma has been that prions lack nucleic acids …

The immunobiology of prion diseases

A Aguzzi, M Nuvolone, C Zhu - Nature Reviews Immunology, 2013 - nature.com
Individuals infected with prions succumb to brain damage, and prion infections continue to
be inexorably lethal. However, many crucial steps in prion pathogenesis occur in lymphatic …

The function of the cellular prion protein in health and disease

JC Watts, MEC Bourkas, H Arshad - Acta neuropathologica, 2018 - Springer
The essential role of the cellular prion protein (PrP C) in prion disorders such as Creutzfeldt–
Jakob disease is well documented. Moreover, evidence is accumulating that PrP C may act …

Strictly co-isogenic C57BL/6J-Prnp−/− mice: A rigorous resource for prion science

M Nuvolone, M Hermann, S Sorce, G Russo… - Journal of Experimental …, 2016 - rupress.org
Although its involvement in prion replication and neurotoxicity during transmissible
spongiform encephalopathies is undisputed, the physiological role of the cellular prion …

The prion protein knockout mouse: a phenotype under challenge

AD Steele, S Lindquist, A Aguzzi - Prion, 2007 - Taylor & Francis
The key pathogenic event in prion disease involves misfolding and aggregation of the
cellular prion protein (PrP). Beyond this fundamental observation, the mechanism by which …

Genome‐wide association studies for discovery of genes involved in asthma

L Akhabir, AJ Sandford - Respirology, 2011 - Wiley Online Library
Asthma is the result of a complex interaction between environmental factors and genetic
variants that confer susceptibility. Studies of the genetics of asthma have previously been …

Prion protein scrapie and the normal cellular prion protein

CJ Atkinson, K Zhang, AL Munn, A Wiegmans, MQ Wei - Prion, 2016 - Taylor & Francis
Prions are infectious proteins and over the past few decades, some prions have become
renowned for their causative role in several neurodegenerative diseases in animals and …

The role of the cellular prion protein in the immune system

JD Isaacs, GS Jackson… - Clinical & Experimental …, 2006 - academic.oup.com
Prion protein (PrP) plays a key role in the pathogenesis of prion diseases. However, the
normal function of the protein remains unclear. The cellular isoform (PrPC) is expressed …

Prion protein regulates iron transport by functioning as a ferrireductase

A Singh, S Haldar, K Horback, C Tom… - Journal of …, 2013 - content.iospress.com
Prion protein (PrP C) is implicated in the pathogenesis of prion disorders, but its normal
function is unclear. We demonstrate that PrP C is a ferrireductase (FR), and its absence …