Results 61 to 70 of about 49,492 (243)

Cerebrospinal fluid biomarkers in E200K genetic Creutzfeldt–Jakob disease: a national study from an endemic Slovak cohort

Frontiers in Neuroscience
BackgroundGenetic Creutzfeldt–Jakob disease (gCJD) is a rare inherited prion disorder caused by pathogenic variants in the PRNP gene. Slovakia represents one of the major endemic regions worldwide, with most genetic cases linked to the E200K variant ...
Dana Zakova, Silvia Koscova, Katarina Melicherova, Alzbeta Janakova, Zuzana Szarvasova, Dana Kosorinova, Martin Stelzer   +6 more
doaj   +1 more source

Pathologic and biochemical characterization of PrPSc from elk with PRNP polymorphisms at codon 132 after experimental infection with the chronic wasting disease agent

BMC Veterinary Research, 2018
Background The Rocky Mountain elk (Cervus elaphus nelsoni) prion protein gene (PRNP) is polymorphic at codon 132, with leucine (L132) and methionine (M132) allelic variants present in the population.
S. Jo Moore, Catherine E. Vrentas, Soyoun Hwang, M. Heather West Greenlee, Eric M. Nicholson, Justin J. Greenlee   +5 more
doaj   +1 more source

Aptamer‐Targeted PrPC Drives Colorectal Cancer Metastasis via a LYN‐STAT3 Complex and Enables Liquid Biopsy Detection

Advanced Science, EarlyView.
The aptamer WHY‐3E identifies PrPC as a CRC driver. Stabilized by USP18, endocytosed PrPC forms a LYN/STAT3 complex, upregulating MSN transcription to promote metastasis. Crucially, WHY‐3E sensitively detects PrPC‐positive circulating exosomes, establishing a robust theoretical foundation for non‐invasive clinical diagnostics.
Chunlin Wang, Hongyu Wu, Chaojing Zheng, Hao Zhang, Xiaoqiu Wu, Jiaqi Wang, Zewen Chang, Jun Xiang, Yunxiao Liu, Chenkai Zhang, Yuliuming Wang, Hao Jiang, Yuchen Zhong, Jun Luo, Ying Chen, NaNa Zhang, Weiyuan Zhang, Ziming Yuan, ChaoXia Zou, Weihong Tan, Meng Wang, Hanqing Hu, Tao Bing, Guiyu Wang   +23 more
wiley   +1 more source

PLGA nanoparticles for oral delivery of prion-specific antigen: a novel approach to chronic wasting disease vaccination

Scientific Reports
Prion diseases, such as chronic wasting disease (CWD), are incurable, fatal neurodegenerative disorders. We have developed a recombinant dimeric deer prion protein (Ddi) vaccine against CWD that has shown promising immune responses when injected ...
Mohamed M. Elsutohy, Dalia Abdelaziz, Chimoné S. Dalton, Byron Kruger, Shirley Phan, Yo-Ching Cheng, Kevin Low, Hanaa Ahmed-Hassan, Hermann M. Schätzl   +8 more
doaj   +1 more source

p300 Degradation by the p53‐SIAH1 Axis Relieves TBK1 Acetylation to Enhance Innate Antiviral Immunity

Advanced Science, EarlyView.
This study identifies p300 as the acetyltransferase that acetylates TBK1 and inhibits its phosphorylation. Activation of the p53‐SIAH1 axis by immune response downregulates p300 expression to sustain innate antiviral immunity. Conditional p300 knockout in alveolar epithelial cells in vivo promotes antiviral responses and suppresses virus replication ...
Huidi Yu, Zhihao Zhan, Xiaoxiang Pan, Xinyue Zhang, Penggang Liu, Jing Sun, Xiulong Xu   +6 more
wiley   +1 more source

A Phase‐Resolved Geometric Deep Learning Framework Maps Structural Determinants of Disease‐Associated Protein Aggregation and Guides Suppressor Design

Advanced Science, EarlyView.
SKALE 2.0 maps disease‐associated protein aggregation as a phase‐resolved structural process, linking mutation‐induced geometric perturbations to nucleation, elongation, and suppressor design. Across neurodegenerative proteins, the framework reveals cryptic aggregation vulnerabilities, separates phase‐concordant and phase‐switching mutations, and ...
Jia Shen Sio, Wei Xuan Wilson Loo, Yan Shan Loo, Wen Xin Tan, Hui Xuan Lim, Huitao Liu, Chen Seng Ng   +6 more
wiley   +1 more source

Inhibition and Formation of Amyloid Fibrils in the Bulk and at the Interface of Biomolecular Condensates

Angewandte Chemie, EarlyView.
In this review, we discuss how biomolecular condensates can inhibit amyloid aggregation in their interior, while still facilitating fibril formation at the interface between the dense and dilute phases, where molecular and mesoscale properties are likely optimal to promote protein aggregation.
Marcell Papp, Chiara Morelli, Sarah Khawaja, Paolo Arosio   +3 more
wiley   +2 more sources

Prions in Yeast [PDF]

Genetics, 2012
AbstractThe concept of a prion as an infectious self-propagating protein isoform was initially proposed to explain certain mammalian diseases. It is now clear that yeast also has heritable elements transmitted via protein. Indeed, the “protein only” model of prion transmission was first proven using a yeast prion.
Susan W, Liebman, Yury O, Chernoff
openaire   +2 more sources

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

ApoE4 lowers age at onset in patients with frontotemporal dementia and tauopathy independent of amyloid‐β copathology

Alzheimer’s & Dementia: Diagnosis, Assessment & Disease Monitoring, 2019
Introduction Apolipoprotein E (ApoE) is the most important genetic risk factor for Alzheimer's disease (AD), with ApoE4 thought to enhance and accelerate amyloid‐β (Aβ) pathology. ApoE4 has recently been described to increase neurodegeneration in a mouse
Carolin Koriath, Tammaryn Lashley, William Taylor, Ronald Druyeh, Athanasios Dimitriadis, Nicola Denning, Julie Williams, Jason D. Warren, Nick C. Fox, Jonathan M. Schott, James B. Rowe, John Collinge, Jonathan D. Rohrer, Simon Mead   +13 more
doaj   +1 more source