Results 1 to 10 of about 10,379,704 (316)

A noninvasive test for human prion disease using hair roots and scalp [PDF]

Scientific Reports
Invasive tests like cerebrospinal fluid (CSF) examination are highly effective for diagnosing human prion disease (HPD). Real-time quaking-induced conversion (RT-QuIC) CSF assay demonstrates 80–90% sensitivity for HPD diagnosis.
Thi-Thu-Trang Dong, Hiroyuki Honda, Akio Akagi, Yasushi Iwasaki, Hitaru Kishida, Tadashi Tsukamoto, Kensaku Kasuga, Hirotsugu Takashima, Tatsuhiro Terada, Kensuke Ikenaka, Takeshi Ikeuchi, Tsuyoshi Mori, Hideki Mochizuki, Kenjiro Ono, Yoshihisa Takiyama, Tsuyoshi Hamaguchi, Hiroyuki Murota, Nobuo Sanjo, Takeshi Fujimoto, Michio Kitayama, Koji Fujita, Motohiro Yukitake, Shinsuke Fujioka, Noriyuki Nishida, Yoshio Tsuboi, Tetsuyuki Kitamoto, Masaki Takao, Masahito Yamada, Hidehiro Mizusawa, Katsuya Satoh   +29 more
doaj   +2 more sources

Aqueous extraction of formalin-fixed paraffin-embedded tissue and detection of prion disease using real-time quaking-induced conversion [PDF]

BMC Research Notes
Objective The goal of the research presented here is to determine if methods previously developed for the aqueous extraction of PrPSc from formalin-fixed paraffin-embedded tissue (FFPET) are applicable to the detection PrPSc by real-time quaking induced ...
Eric M. Nicholson, Justin J. Greenlee, Soyoun Hwang   +2 more
doaj   +2 more sources

Convergent generation of atypical prions in knockin mouse models of genetic prion disease [PDF]

The Journal of Clinical Investigation
Most cases of human prion disease arise due to spontaneous misfolding of WT or mutant prion protein, yet recapitulating this event in animal models has proven challenging. It remains unclear whether spontaneous prion generation can occur within the mouse
Surabhi Mehra, Matthew E.C. Bourkas, Lech Kaczmarczyk, Erica Stuart, Hamza Arshad, Jennifer K. Griffin, Kathy L. Frost, Daniel J. Walsh, Surachai Supattapone, Stephanie A. Booth, Walker S. Jackson, Joel C. Watts   +11 more
doaj   +2 more sources

The PINK1/Parkin pathway of mitophagy exerts a protective effect during prion disease. [PDF]

PLoS ONE
The PINK1/Parkin pathway of mitophagy has been implicated in the pathogenesis of Parkinson's disease. In prion diseases, a transmissible neurodegenerative disease caused by the misfolded and infectious prion protein (PrPSc), expression of both PINK1 and ...
Anne Ward, Forrest Jessop, Robert Faris, Jason Hollister, Daniel Shoup, Brent Race, Catharine M Bosio, Suzette A Priola   +7 more
doaj   +2 more sources

PINK1-parkin-mediated neuronal mitophagy deficiency in prion disease

Cell Death and Disease, 2022
A persistent accumulation of damaged mitochondria is part of prion disease pathogenesis. Normally, damaged mitochondria are cleared via a major pathway that involves the E3 ubiquitin ligase parkin and PTEN-induced kinase 1 (PINK1) that together initiate ...
Jie Li, Mengyu Lai, Xixi Zhang, Zhiping Li, Dongming Yang, Mengyang Zhao, Dongdong Wang, Z. Sun, Sharjeel Ehsan, Wen Li, Hongli Gao, Deming Zhao, Lifeng Yang   +12 more
semanticscholar   +1 more source

Neuroinflammation in Prion Disease

International Journal of Molecular Sciences, 2021
Neuroinflammation, typically manifest as microglial activation and astrogliosis accompanied by transcriptomic alterations, represents a common hallmark of various neurodegenerative conditions including prion diseases.
Bei Li, Meiling Chen, Caihong Zhu
semanticscholar   +1 more source

Microglia deficiency accelerates prion disease but does not enhance prion accumulation in the brain

bioRxiv, 2021
Prion diseases are transmissible, neurodegenerative disorders associated with misfolding of the prion protein. Previous studies show that reduction of microglia accelerates CNS prion disease and increases the accumulation of prions in the brain ...
Barry M. Bradford, L. McGuire, D. Hume, Clare Pridans, N. Mabbott   +4 more
semanticscholar   +1 more source

Transmission of Raccoon-Passaged Chronic Wasting Disease Agent to White-Tailed Deer

Viruses, 2022
The transmission characteristics of prion diseases are influenced by host prion protein sequence and, therefore, the host species. Chronic wasting disease (CWD), a prion disease of cervids, has widespread geographical distribution throughout North ...
Eric D. Cassmann, Alexis J. Frese, S. Jo Moore, Justin J. Greenlee   +3 more
doaj   +1 more source

Interspecies Transmission from Pigs to Ferrets of Antigenically Distinct Swine H1 Influenza A Viruses with Reduced Reactivity to Candidate Vaccine Virus Antisera as Measures of Relative Zoonotic Risk

Viruses, 2022
During the last decade, endemic swine H1 influenza A viruses (IAV) from six different genetic clades of the hemagglutinin gene caused zoonotic infections in humans.
J. Brian Kimble, Carine K. Souza, Tavis K. Anderson, Zebulun W. Arendsee, David E. Hufnagel, Katharine M. Young, Nicola S. Lewis, C. Todd Davis, Sharmi Thor, Amy L. Vincent Baker   +9 more
doaj   +1 more source

Prion protein lowering is a disease-modifying therapy across prion disease stages, strains and endpoints

bioRxiv, 2020
Lowering of prion protein (PrP) expression in the brain is a genetically validated therapeutic hypothesis in prion disease. We recently showed that antisense oligonucleotide (ASO)-mediated PrP suppression extends survival and delays disease onset in ...
E. Minikel, H. Zhao, Jason Lê, J. O’Moore, Rose Pitstick, Samantha Graffam, G. Carlson, M. Kavanaugh, J. Kriz, J. B. Kim, Jiyan Ma, H. Wille, J. Aiken, D. McKenzie, K. Doh-ura, M. Beck, R. O’Keefe, J. Stathopoulos, T. Caron, S. Schreiber, J. Carroll, H. Kordasiewicz, D. Cabin, S. M. Vallabh   +23 more
semanticscholar   +1 more source