Results 51 to 60 of about 14,426 (178)

PolyG Fibrils Coalesce Into Nuclear Ribbons That Engage Proteostasis Machinery in Neuronal Intranuclear Inclusion Disease

Advanced Science, EarlyView.
In NIID, expanded NOTCH2NLC repeats give rise to nuclear polyG inclusions. Tracer‐guided in situ cryo‐electron tomography enables cross‐scale structural analysis from mouse brain to native neuronal nuclei, revealing dense‐core/peripheral‐halo inclusions built from compact polyG ribbons.
Hui Dong, Yongcheng Pan, Zhiyao Tang, Yuxuan Yao, Guicong Zhang, Junpu Wang, Haonan Xiao, Yun Tian, Beisha Tang, Dan Li, Qiang Guo, Ruijun Tian, Qiong Liu, Cong Liu   +13 more
wiley   +1 more source

Preserving Proton Resolution Under Cryogenic Dynamic Nuclear Polarization for Biomolecular Solid‐State NMR

Angewandte Chemie, EarlyView.
High‐resolution proton detection is preserved under cryogenic dynamic nuclear polarization (DNP) by combining fast MAS, extensive deuteration and selective methyl protonation. This strategy enables proton‐detected structural studies of biomolecular solids with enhanced sensitivity under DNP conditions.
James Tolchard, Tanguy Le Marchand, Nadia El Mammeri, Kshama Sharma, Ajit Kumar Bishoyi, Mélanie Berbon, Kristaps Jaudzems, Dorothea Wisser, Asen Daskalov, Sven J. Saupe, Jan Stanek, Zhiyu Sun, Olivier Ouari, Birgit Habenstein, Anne Lesage, Guido Pintacuda, Antoine Loquet   +16 more
wiley   +2 more sources

Comparing the Folds of Prions and Other Pathogenic Amyloids

Pathogens, 2018
Pathogenic amyloids are the main feature of several neurodegenerative disorders, such as Creutzfeldt–Jakob disease, Alzheimer’s disease, and Parkinson’s disease. High resolution structures of tau paired helical filaments (PHFs), amyloid-
José Miguel Flores-Fernández, Vineet Rathod, Holger Wille   +2 more
doaj   +1 more source

A Unifying Thermodynamic Model for Phase Separation and Aging of Biopolymers

Advanced Science, EarlyView.
Phase separation and aging of intrinsically disordered proteins are placed in a unifying framework. A thermodynamically consistent time‐dependent version of associating‐polymer theory shows how the processes are intricately coupled. Assuming aging to occur through interacting sites resulting from reversible conformational transitions, the model ...
Jasper J. Michels, Joana Caria, Edward A. Lemke   +2 more
wiley   +1 more source

Retention of prions in the polychaete Hediste diversicolor and black soldier fly, Hermetia illucens, larvae after short-term experimental immersion and feeding with brain homogenate from scrapie infected sheep

Heliyon
Finding alternative protein and lipid sources for aquafeeds is crucial for the sustainable growth of fed aquaculture. Upcycling industrial side streams and byproducts using extractive species can reduce waste and help reduce the sector's dependence on ...
Sylvie L. Benestad, Linh Tran, Arne M. Malzahn, Nina S. Liland, Ikram Belghit, Andreas Hagemann   +5 more
doaj   +1 more source

RSF1‐Dependent PAR Turnover Promotes 53BP1 Liquid Condensate Formation at DNA Damage Sites

Advanced Science, EarlyView.
At sites of DNA damage, RSF1 recruits PARG to accelerate PAR turnover, triggering a switch from PAR‐driven condensates to 53BP1 condensates. This condensate transition enables p53‐dependent gene transcription and coordinates the DNA damage response.
Yungyeong Heo, Yonghyeon Kim, Nangyeong Hwang, Gwanho Sung, Sujin Jo, Yeonhu Lee, Ho‐Chul Kang, Jae‐Ho Lee, Yong Won Choi, Hyeseong Cho, Sunwoo Min   +10 more
wiley   +1 more source

Atypical Scrapie Prions from Sheep and Lack of Disease in Transgenic Mice Overexpressing Human Prion Protein

Emerging Infectious Diseases, 2013
Public and animal health controls to limit human exposure to animal prions are focused on bovine spongiform encephalopathy (BSE), but other prion strains in ruminants may also have zoonotic potential.
Jonathan D.F. Wadsworth, Susan Joiner, Jacqueline M. Linehan, Anne Balkema-Buschmann, John Spiropoulos, Marion M. Simmons, Peter C. Griffiths, Martin H. Groschup, James Hope, Sebastian Brandner, Emmanuel A. Asante, John Collinge   +11 more
doaj   +1 more source

Tau Aggregate Imaging and Transcriptomics of Alzheimer's Disease Brain at Different Stages of Disease

Advanced Science, EarlyView.
The protein aggregates and gene expression in the middle temporal gyrus (MTG) and somatosensory cortex (SOM) of the postmortem brains of 13 Alzheimer's disease patients were studied in detail, revealing that small hyperphosphorylated tau aggregates increase with Braak stage driven by microglial inflammation.
Elizabeth A. English, Dorothea Böken, Matthew W. Cotton, Matthew R. Cheetham, Johanna S. Jackson, Georg Meisl, John S. H. Danial, Paul M. Matthews, Nurun N. Fancy, David Klenerman   +9 more
wiley   +1 more source

14‐3‐3γ Protects Against Postoperative Cognitive Dysfunction by Regulating Tau Thr205 Phosphorylation and Synaptic Integrity

Advanced Science, EarlyView.
Integrative multi‐cohort analyses identify 14‐3‐3γ as a biomarker and regulator of cognitive vulnerability. Experimental studies show that 14‐3‐3γ loss is associated with Tau Thr205 phosphorylation, synaptic dysfunction, and cognitive impairment, while genetic overexpression or pharmacological stabilization of 14‐3‐3γ confers protective effects in ...
Shouqiang Zhu, Xue Han, Heng Zhang, Chang Ye, Tianjiao Xia, Xiaoping Gu   +5 more
wiley   +1 more source

Accelerated high fidelity prion amplification within and across prion species barriers.

PLoS Pathogens, 2008
Experimental obstacles have impeded our ability to study prion transmission within and, more particularly, between species. Here, we used cervid prion protein expressed in brain extracts of transgenic mice, referred to as Tg(CerPrP), as a substrate for ...
Kristi M Green, Joaquín Castilla, Tanya S Seward, Dana L Napier, Jean E Jewell, Claudio Soto, Glenn C Telling   +6 more
doaj   +1 more source