Results 61 to 70 of about 2,165,687 (231)

Seeding and transgenic overexpression of alpha‐synuclein triggers dendritic spine pathology in the neocortex

EMBO Molecular Medicine, 2017
Although misfolded and aggregated α‐synuclein (α‐syn) is recognized in the disease progression of synucleinopathies, its role in the impairment of cortical circuitries and synaptic plasticity remains incompletely understood.
Sonja Blumenstock, Eva F Rodrigues, Finn Peters, Lidia Blazquez‐Llorca, Felix Schmidt, Armin Giese, Jochen Herms   +6 more
doaj   +1 more source

Structural Polymorphism of polyG Inclusions Revealed by In Situ Cryo‐Electron Tomography

Advanced Science, EarlyView.
Correlative cryo‐electron tomography in primary cortical neurons and NIID mouse brain tissue reveals that polyG inclusions are interconnected ribbon‐like assemblies rather than canonical amyloid fibrils. Multiple compartment‐specific ribbon states show distinct 26S proteasome accessibility, while cytoplasmic ribbons contact and deform ER‐like ...
Yunwen Qian, Yalan Wan, Chen Chen, Xiaoyu Zheng, Wenjing Du, Junhan Yang, Zhihao Quan, Biyu Yang, Jiaxi Yu, Jie Zheng, Zhaoxia Wang, Jianwen Deng, Qiang Guo   +12 more
wiley   +1 more source

SERPINA3/SerpinA3n: a novel promising therapeutic target in prion diseases [PDF]

, 2021
Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), belong to a class of fatal neurodegenerative disorders characterized by vacuolation and neuronal loss in the brain paralleled by cognitive and motor impairments.
Colini Baldeschi, Arianna
core  

Regulation of MicroRNAs-Mediated Autophagic Flux: A New Regulatory Avenue for Neurodegenerative Diseases With Focus on Prion Diseases

Frontiers in Aging Neuroscience, 2018
Prion diseases are fatal neurological disorders affecting various mammalian species including humans. Lack of proper diagnostic tools and non-availability of therapeutic remedies are hindering the control strategies for prion diseases. MicroRNAs (miRNAs)
Syed Zahid Ali Shah, Deming Zhao, Tariq Hussain, Naveed Sabir, Lifeng Yang   +4 more
doaj   +1 more source

A Phosphorylation‐Induced Micellization Switch in the Low‐Complexity Domain of TDP‐43

Advanced Science, EarlyView.
Phosphorylation of TAR DNA‐binding protein's 43 kDa (TDP‐43) low‐complexity domain by casein kinase 1 delta (CK1δ) acts as a molecular switch, redirecting its self‐assembly from macroscopic phase separation toward finite‐sized, spherical block‐copolymer micelles of ∼30 nm.
Rodrigo F. Dillenburg, Anastasia Lopatina, Hao Ruan, Tom Scheidt, Simone Mosna, Emre Pekbilir, Julia Bieber, Frank Schäfer‐Depoix, Katharina Landfester, Carla Schmidt, Martin M. Möckel, Svenja Morsbach, Friederike Schmid, Dorothee Dormann, Lukas Stelzl, Martin Girard, Edward A. Lemke   +16 more
wiley   +1 more source

Development of a sensitive cell culture system to assess prion infectivity and the efficacy of prion decontamination technologies

, 2012
Creutzfeldt-Jakob disease (CJD) can be iatrogenically transmitted during transplants, grafts and transfusions from CJD infected donors and also contaminated surgical instruments.
Secker, Thomas
core   +1 more source

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

Effect of enzymatic deimination on the conformation of recombinant prion protein [PDF]

, 2009
Deimination is the post-translational conversion of arginine residues to citrulline. It has been implicated as a causative factor in autoimmune diseases such as multiple sclerosis and rheumatoid arthritis and more recently, as a marker of ...
Oxley, David, Meersman, Filip, Webster, Judith, Young, Duncan S., Dear, Denise V., Bronstein, Igor, Gill, Andy   +6 more
core  

Prion protein in the cerebrospinal fluid of healthy and naturally scrapie-affected sheep [PDF]

, 2006
The aim of this study was to characterize the cerebrospinal fluid (CSF) prion protein (PrP) of healthy and naturally scrapie-affected sheep. The soluble form of CSF PrPC immunoblotted with an anti-octarepeat and an anti-C terminus mAb showed two isoforms
Vé Ronique Gayrard, Moudjou, Mohammed, Gayrard-Troy, Véronique, V., Pierre-Louis Toutain, Mohammed Moudjou, Chantal Imbs, Viguié, Catherine, Toutain, Pierre-Louis, Imbs, Chantal, Moudjou, Mohammed, M., Catherine Viguié, Nicole Picard-Hagen, Picard-Hagen, Nicole, Gayrard, Véronique   +13 more
core   +1 more source

Loss of PIKfyve drives the spongiform degeneration in prion diseases

EMBO Molecular Medicine, 2021
Brain‐matter vacuolation is a defining trait of all prion diseases, yet its cause is unknown. Here, we report that prion infection and prion‐mimetic antibodies deplete the phosphoinositide kinase PIKfyve—which controls endolysosomal maturation—from mouse
Asvin K K Lakkaraju, Karl Frontzek, Emina Lemes, Uli Herrmann, Marco Losa, Rajlakshmi Marpakwar, Adriano Aguzzi   +6 more
doaj   +1 more source