Results 91 to 100 of about 78,455 (315)

The most problematic symptoms of prion disease - an analysis of carer experiences [PDF]

, 2019
Objectives: Prion diseases are rare dementias that most commonly occur sporadically, but can be inherited or acquired, and for which there is no cure.
Collinge, John, Ford, Liz, Gorham, Michele, Mead, Simon, Robinson, Kathy, Rudge, Peter   +5 more
core   +2 more sources

Prion disease genetics [PDF]

European Journal of Human Genetics, 2006
Prion diseases have stimulated intense scientific scrutiny since it was proposed that the infectious agent was devoid of nucleic acid. Despite this finding, genetics has played a key role in understanding the pathobiology and clinical aspects of prion disease through the effects of a series of polymorphisms and mutations in the prion protein gene (PRNP)
openaire   +2 more sources

EGR1 Nuclear Condensates Promote Renal Cyst Development in Polycystic Kidney Disease

Exploration, EarlyView.
ABSTRACT Autosomal dominant polycystic kidney disease (ADPKD) is marked by aberrant cell proliferation driven by cAMP‐PKA and MAPK signaling pathways. EGR1, a transcription factor directly activated by the above two pathways, is critical in the over‐proliferation of tumor cells, which share similarities with cystic epithelial cells in ADPKD. This study
Chaoqun Ren, Zhaoxu Wu, Min Li, Yaqin Du, Hong Zhou, Yazhu Quan, Mengyao Xiong, Yiming Wang, Zhiwei Qiu, Shuai Zhu, Xiaowei Li, Jinzhao He, Cai Gao, Hui Cai, Tingting Li, Baoxue Yang   +15 more
wiley   +1 more source

Helices 2 and 3 are the initiation sites in the PrPc -> PrPsc transition

, 2012
It is established that prion protein is the sole causative agent in a number of diseases in humans and animals. However, the nature of conformational changes that the normal cellular form PrPC undergoes in the conversion process to a self-replicating ...
Chen, Jie, Thirumalai, Devarajan
core   +1 more source

Methods for differentiating prion types in food-producing animals [PDF]

, 2015
Prions are an enigma amongst infectious disease agents as they lack a genome yet confer specific pathologies thought to be dictated mainly, if not solely, by the conformation of the disease form of the prion protein (PrPSc).
Gough, Kevin C., Ives, Sarah E., Maddison, Ben C., Rees, Helen C.   +3 more
core   +2 more sources

TSE pathogenesis in cattle and sheep [PDF]

, 2008
Many studies have been undertaken in rodents to study the pathogenesis of transmissible spongiform encephalopathies (TSE). Only a few studies have focused on the pathogenesis of bovine spongiform encephalopathy (BSE) and scrapie in their natural hosts ...
Bossers, A., Keulen, L.J.M., van, Zijderveld, F.G., van   +2 more
core   +3 more sources

Plasmonics‐Enhanced Characterization of Cervid PrP (87–114) Fragment Aggregates in Solution

Journal of Biophotonics, EarlyView.
Multimodal nanophotonics platform combining plasmon‐enhanced imaging, label‐free SERS, and optical staining reveals new insights into the aggregation of the cervid PrP (87–114) fragment, an amyloidogenic sequence linked to chronic wasting disease susceptibility.
Shinki Midha, Aishwarya Sriraman, Lyudmyla Dorosh, Holger Wille, Maria Stepanova   +4 more
wiley   +1 more source

Transmission and Characterization of Creutzfeldt–Jakob Disease and Chronic Wasting Disease in the North American Deer Mouse

Viruses
Prion transmission into rodents is essential for understanding prion strains. However, it is often limited by a “species barrier” that makes transmission challenging and complicates the study of animal and human prion diseases. Here, we report that North
Jennifer Myskiw, Lise Lamoureux, Kathy Frost, Rebecca Fox, Jessy A. Slota, Gordon Mitchell, Ben A. Bailey-Elkin, Stephanie A. Booth   +7 more
doaj   +1 more source

MicroRNA Alterations in the Brain and Body Fluids of Humans and Animal Prion Disease Models: Current Status and Perspectives

Frontiers in Aging Neuroscience, 2018
Prion diseases are transmissible progressive neurodegenerative conditions characterized by rapid neuronal loss accompanied by a heterogeneous neuropathology, including spongiform degeneration, gliosis and protein aggregation.
Eirini Kanata, Katrin Thüne, Konstantinos Xanthopoulos, Isidre Ferrer, Isidre Ferrer, Isidre Ferrer, Dimitra Dafou, Inga Zerr, Theodoros Sklaviadis, Franc Llorens, Franc Llorens, Franc Llorens   +11 more
doaj   +1 more source

Differentiation of ruminant transmissible spongiform encephalopathy isolate types, including bovine spongiform encephalopathy and CH1641 scrapie [PDF]

, 2010
With increased awareness of the diversity of transmissible spongiform encephalopathy (TSE) strains in the ruminant population, comes an appreciation of the need for improved methods of differential diagnosis. Exposure to bovine spongiform encephalopathy (
Bossers, A., Jacobs, J.G., Keulen, L.J.M., van, Langeveld, J.P.M., Sauer, M., Tang, Y.   +5 more
core   +2 more sources