Results 81 to 90 of about 928,578 (204)

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

Additional file 1: of Replication of multiple system atrophy prions in primary astrocyte cultures from transgenic mice expressing human α-synuclein

, 2019
Figure S1. Isolation and characterization of astrocyte cultures from transgenic (Tg) mice. Figure S2. Human α-synuclein expression levels in astrocyte cultures from transgenic (Tg) mice. Figure S3.
Carlson, George A, Prusiner, Stanley B, Kurt Giles (195746), Stanley Prusiner (4605775), Krejciova, Zuzana, Giles, Kurt, Zuzana Krejciova (4605781), George Carlson (4605790)   +7 more
core   +1 more source

Genetic variation in complex traits in transgenic α-synuclein strains of Caenorhabditis elegans

, 2020
Different genetic backgrounds can modify the effect of mutated genes. Human α-synuclein (SNCA) gene encodes α-synuclein, and its oligomeric complexes accumulate with age and mediate the disruption of cellular homeostasis, resulting in the neuronal death ...
Sterken, M.G., Harvey, S., van Sluijs, L., Nie, Y., Wang, Y., Kammenga, J.E.   +5 more
core   +1 more source

Oxidative stress in genetic mouse models of Parkinson's disease [PDF]

, 2012
There is extensive evidence in Parkinson’s disease of a link between oxidative stress and some of the monogenically inherited Parkinson’s disease-associated genes.
Bentea, Eduard, Michotte, Yvette, Varcin, Mustafa, Sarre, Sophie   +3 more
core   +1 more source

Trafficking Deficiency of TMEM175 Variants in Parkinson's Disease Pathogenesis and the Prospects of Precision Medicine

Advanced Science, EarlyView.
This study identifies that the PD‐associated TMEM175‐L156P variant disrupts lysosomal ion channel trafficking by causing aberrant endoplasmic reticulum retention. A “chaperone–agonist” bifunctional small molecule restores TMEM175‐L156P lysosomal localization and channel function, thereby alleviating PD‐relevant cellular phenotypes and highlighting a ...
Ting Luo, Yu He, Shuyao Li, Haoyu Guan, Ruili Cui, Yu Shi, Zhongwen Jiang, Siyu Wang, Xuan Li, Jiyuan Li, Mei Hu, Yupeng Zhou, Beisha Tang, Yanyan Zhang, Zhaobing Gao, Yu Zhou, Zhenhua Liu, Ping Li   +17 more
wiley   +1 more source

α-Synuclein strain propagation is independent of cellular prion protein expression in a transgenic synucleinopathy mouse model.

PLoS Pathogens
The cellular prion protein, PrPC, has been postulated to function as a receptor for α-synuclein, potentially facilitating cell-to-cell spreading and/or toxicity of α-synuclein aggregates in neurodegenerative disorders such as Parkinson's disease ...
Raphaella W L So, Genki Amano, Erica Stuart, Aeen Ebrahim Amini, Adriano Aguzzi, Graham L Collingridge, Joel C Watts   +6 more
doaj   +1 more source

Recombinant human neuroblastoma sh-sy5y cells overexpressing α-synuclein form amyloid aggregates in seed-dependent and seed-independent manners [PDF]

Alpha-synuclein (α-Syn) has prion-like properties and is one of the causative proteins of Parkinson’s disease (PD) and dementia with Lewy bodies (DLB).
Yoshiharu OKUNO, Ryohei KONO, Haruki FUJIHIRA, Hirotoshi UTSUNOMIYA   +3 more
core   +1 more source

Systematic Multi‐Level Analyses Decode the Arthritis‐Neurodegeneration Axis With In Vivo Validation

Advanced Science, EarlyView.
Arthritis and neurodegeneration are usually studied as separate disorders, but this study connects them through population evidence, genetic inference, transcriptomic mapping, and mouse models. It highlights RNF40 as a context‐dependent joint‐brain candidate, induced in inflammatory joints yet functionally linked to dopamine‐neuron vulnerability ...
Jinwen Wang, Wenhui Xie, Lei Yang, Zhengrong Wang, Yi Huang, Peng Huang, Yuan Liu, Jun Hu   +7 more
wiley   +1 more source

MJF-14 proximity ligation assay detects early non-inclusion alpha-synuclein pathology with enhanced specificity and sensitivity

npj Parkinson's Disease
α-Synuclein proximity ligation assay (PLA) has proved a sensitive technique for detection of non-Lewy body α-synuclein aggregate pathology. Here, we describe the MJF-14 PLA, a new PLA towards aggregated α-synuclein with unprecedented specificity, using ...
Nanna Møller Jensen, YuHong Fu, Cristine Betzer, Hongyun Li, Sara Elfarrash, Ali H. Shaib, Donatus Krah, Zagorka Vitic, Lasse Reimer, Hjalte Gram, Vladimir Buchman, Mark Denham, Silvio O. Rizzoli, Glenda M. Halliday, Poul Henning Jensen   +14 more
doaj   +1 more source

The E46K mutation modulates α-synuclein prion replication in transgenic mice

PLoS Pathogens, 2022
In multiple system atrophy (MSA), the α-synuclein protein misfolds into a self-templating prion conformation that spreads throughout the brain, leading to progressive neurodegeneration.
Sara A. M. Holec, Jisoo Lee, Abby Oehler, Lyn Batia, Aryanna Wiggins-Gamble, Jeffrey Lau, Felicia K. Ooi, Gregory E. Merz, Man Wang, Daniel A. Mordes, Steven H. Olson, Amanda L. Woerman   +11 more
doaj