Results 121 to 130 of about 97,942 (322)

Epidemiology of progressive intellectual and neurological deterioration in UK children

Developmental Medicine &Child Neurology, EarlyView.
This study of PIND in UK children was carried out via the British Paediatric Surveillance Unit from 1997 to 2024. It identified six cases of vCJD. 2367 children had other diagnoses explain their deterioration. There were 259 other diseases in the diagnosed group.
Christopher M. Verity, Polly J. Maunder, Anne Marie Winstone, Suvankar Pal   +3 more
wiley   +1 more source

Chronic wasting disease as a model for human prion therapy

Prion
Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are fatal neurodegenerative disorders that result from abnormally folded prion proteins. These disorders can be sporadic, acquired, or genetic.
Michael Bordonaro
doaj   +1 more source

Region-specific glial homeostatic signature in prion diseases is replaced by a uniform neuroinflammation signature, common for brain regions and prion strains with different cell tropism

, 2020
Natallia Makarava, Jennifer C. Chang, Kara Molesworth, Ilia V. Baskakov   +3 more
openalex   +1 more source

Prion-like behavior of MAVS in RIG-I signaling [PDF]

, 2011
Eva Marie Y. Moresco, Diantha La Vine, Bruce Beutler   +2 more
openalex   +1 more source

Co‐localization of tau and TDP‐43 after extracellular vesicle delivery to cells

The FEBS Journal, EarlyView.
Extracellular vesicles (EVs) derived from donor cells transfected with EGFP–2N4R‐tau or mCherry‐wtTDP‐43 were taken up by recipient cells, leading to cytosolic co‐localization of tau and TDP‐43. Molecular modeling revealed that tau and TDP‐43 directly interact through hydrogen bonding, suggesting a mechanistic link underlying their co‐pathology ...
Farhang Aliakbari, Kathryn Volkening, Zahra Nayeri, Aysegul Yucel Polat, Neil Donison, Jacqueline Palik, Michael J. Strong   +6 more
wiley   +1 more source

Prion seeding activity in DNA extractions: implications for laboratory biosafety

Prion
Infectious prions (PrPSc) are largely resistant to proteolytic digestion, including proteinase K (PK) digestion. While nucleic acid extracts are generally considered non-infectious from a classical microbiology context (i.e.
Sarah C. Gresch, Tamara Morrill, Maddy Ellis-Cramer, Maria Arifin, Lexi E. Frank, Jason C. Bartz, Marc D. Schwabenlander, Tiffany M. Wolf, Gordon B. Mitchell, Jiewen Guan, Peter A. Larsen   +10 more
doaj   +1 more source

MAVS oligomerization drives a faster and more efficient antiviral signaling activation at peroxisomes compared to mitochondria

The FEBS Journal, EarlyView.
Cells rely on mitochondria and peroxisomes to trigger antiviral defenses via the protein MAVS. This manuscript demonstrates that the robust production of antiviral effectors resulting from MAVS activation at peroxisomes is faster than at mitochondria due to a swifter oligomerization of this protein at peroxisomal membranes. These results underscore the
Bruno Ramos, Jéssica Sarabando, Mariana Marques, Jonathan C. Kagan, Daniela Ribeiro   +4 more
wiley   +1 more source

La fantástica historia de la increíble prion [PDF]

, 2020
Walter Ledermann D
openalex   +1 more source

Redox environment modulates aggregation of ataxin‐3 in vitro — Implications for drug screening of cysteine‐rich proteins

The FEBS Journal, EarlyView.
Redox environment modulates in vitro aggregation of Ataxin‐3, the protein implicated in spinocerebellar ataxia type 3. Reducing conditions stabilize native monomers and prevent aggregation, whereas oxidative conditions promote the formation of non‐native conformers and disulfide‐linked oligomers within the Josephin domain (JD).
Martyna Podlasiak, Martina Sollazzo, Elisa Monaca, Raffaele Sabbatella, Maria Agnese Morando, Oleg Chertkov, Laura Puglisi, Martina Mascellino, Anna Fricano, Sandra Macedo‐Ribeiro, Caterina Alfano   +10 more
wiley   +1 more source

Amyloid properties of the yeast cell wall protein Toh1 and its interaction with prion proteins Rnq1 and Sup35 [PDF]

, 2018
Aleksandra Sergeeva, Julia V. Sopova, T.A. Belashova, V.A. Siniukova, Angelina V. Chirinskaite, Galkin Ap, S. P. Zadorsky   +6 more
openalex   +1 more source