Results 31 to 40 of about 10,379,704 (316)
Neuroinflammation, Microglia, and Cell-Association during Prion Disease
Viruses, 2019 Prion disorders are transmissible diseases caused by a proteinaceous infectious agent that can infect the lymphatic and nervous systems. The clinical features of prion diseases can vary, but common hallmarks in the central nervous system (CNS) are ...
J. A. Carroll, B. Chesebro
semanticscholar +1 more source
Veterinary Research, 2018 In animal prion diseases, including bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease in cervids, and scrapie in sheep and goats, a disease-associated isoform of prion protein (PrPd) accumulates in the brains of affected animals ...
Kohtaro Miyazawa +5 more
doaj +1 more source
A cationic tetrapyrrole inhibits toxic activities of the cellular prion protein [PDF]
, 2016 Prion diseases are rare neurodegenerative conditions associated with the conformational conversion of the cellular prion protein (PrPC) into PrPSc, a self-replicating isoform (prion) that accumulates in the central nervous system of affected individuals.
Biasini, Emiliano +16 more
core +2 more sources
Kuru, the First Human Prion Disease
Viruses, 2019 Kuru, the first human prion disease was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). In this review, we summarize the history of this seminal discovery, its anthropological background, epidemiology, clinical picture, neuropathology ...
P. Liberski +3 more
semanticscholar +1 more source
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 +5 more
core +2 more sources
Human stem cell-derived astrocytes replicate human prions in a PRNP genotype-dependent manner. [PDF]
, 2017 Prions are infectious agents that cause neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD). The absence of a human cell culture model that replicates human prions has hampered prion disease research for decades.
Alibhai, James +9 more
core +1 more source
Age at onset in genetic prion disease and the design of preventive clinical trials.
Neurology, 2019 OBJECTIVE To determine whether preventive trials in genetic prion disease could be designed to follow presymptomatic mutation carriers to onset of disease.
E. Minikel +31 more
semanticscholar +1 more source
Shortest known prion protein allele in highly BSE-susceptible lemurs [PDF]
, 2000 We describe the shortest prion protein allele known to date. Surprisingly, it is found as a polymorphism exactly in a species (prosimian lemurs) which seems highly susceptible to oral infection with BSE-derived prions. The truncation of the prion protein
Gilch, S. +2 more
core +1 more source
Proceedings of the National Academy of Sciences of the United States of America, 2019 Significance Human prion disease is a rapidly fatal and incurable neurodegenerative disease. Reduction of prion protein in the brain is a well-supported therapeutic hypothesis, and antisense oligonucleotides with this mechanism of action are currently in
S. M. Vallabh +15 more
semanticscholar +1 more source
Genetic Factors Contributing to the Susceptibility of Development of Prion Diseases [PDF]
, 2017 This paper won an honorable mention writing flag award in the research category. Claire Culbertson, writing for Katherine Bruner’s BIO 325L class, “Lab Experience in Genetics”.Bruner, KatherineUndergraduate ...
Culbertson, Claire
core +1 more source