Results 71 to 80 of about 33,090 (271)

Prion protein E219K polymorphism: from the discovery of the KANNO blood group to interventions for human prion disease

Frontiers in Neurology
KANNO is a new human blood group that was recently discovered. The KANNO antigen shares the PRNP gene with the prion protein and the prion protein E219K polymorphism determines the presence or absence of the KANNO antigen and the development of anti ...
Si-Si Wang, Zhao-Li Meng, Yi-Wen Zhang, Yi-Shuang Yan, Ling-Bo Li   +4 more
doaj   +1 more source

Healthy goats naturally devoid of prion protein

Veterinary Research, 2012
Prion diseases such as scrapie in small ruminants, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in man, are fatal neurodegenerative disorders.
Benestad Sylvie L, Austbø Lars, Tranulis Michael A, Espenes Arild, Olsaker Ingrid   +4 more
doaj   +1 more source

Disparate Modes of Evolution Shaped Modern Prion (PRNP) and Prion-Related Doppel (PRND) Variation in Domestic Cattle. [PDF]

PLoS ONE, 2016
Previous investigations aimed at determining whether the mammalian prion protein actually facilitates tangible molecular aspects of either a discrete or pleiotropic functional niche have been debated, especially given the apparent absence of overt ...
Brian W Brunelle, Allison M O'Grady, Eric M Nicholson, Christopher M Seabury   +3 more
doaj   +1 more source

Prions induce an early Arc response and a subsequent reduction in mGluR5 in the hippocampus

Neurobiology of Disease, 2022
Synapse dysfunction and loss are central features of neurodegenerative diseases, caused in part by the accumulation of protein oligomers. Amyloid-β, tau, prion, and α-synuclein oligomers bind to the cellular prion protein (PrPC), resulting in the ...
Daniel Ojeda-Juárez, Jessica A. Lawrence, Katrin Soldau, Donald P. Pizzo, Emily Wheeler, Patricia Aguilar-Calvo, Helen Khuu, Joy Chen, Adela Malik, Gail Funk, Percival Nam, Henry Sanchez, Michael D. Geschwind, Chengbiao Wu, Gene W. Yeo, Xu Chen, Gentry N. Patrick, Christina J. Sigurdson   +17 more
doaj   +1 more source

Developmental expression of the prion protein gene in glial cells

Neuron, 1995
Replication of prions is dependent on the presence of the host protein PrPc. During the course of disease, PrPc is converted into an abnormal isoform, PrPSc, which accumulates in the brain. Attempts to identify the cell type(s) in which prion replication and PrP conversion occur have reached conflicting results.
Moser M, Colello RJ, Pott U, Oesch B
openaire   +3 more sources

Inherited prion disease with A117V mutation of the prion protein gene: a novel Hungarian family [PDF]

Journal of Neurology, Neurosurgery & Psychiatry, 2001
Three members of a family with inherited prion disease are reported. One additional family member had a progressive neurological disease without details. Two developed symptoms of ataxia, dementia, myoclonus, rigidity, and hemiparesis, and one had a different phenotype with the combination of lower motor neuron deficit, parkinsonism, intellectual ...
G G, Kovács, C, Ertsey, C, Majtényi, I, Jelencsik, L, László, H, Flicker, L, Strain, I, Szirmai, H, Budka   +8 more
openaire   +2 more sources

A Skull Bone Marrow‐to‐Brain Axis Links Osteoblastic Activity to Myeloid Cell Trafficking, Cerebral Blood Flow, and Cognition in Alzheimer's Progression

Advanced Science, EarlyView.
This study reveals that Alzheimer's disease–linked APP expression in bone‐forming cells drives skull bone marrow remodeling and alters its vascular connections to the brain. These changes disrupt immune cell trafficking, cerebral blood flow, and cognition. Targeting bone marrow macrophages restores brain function, highlighting a previously unrecognized
Lei Xiong, Dong Sun, Hao‐Han Guo, Daehoon Lee, Zhipeng Liu, Lin Mei, Wen‐Cheng Xiong   +6 more
wiley   +1 more source

Fatal Familial Insomnia, a Prion Disease with a Mutation at Codon 178 of the Prion Protein Gene [PDF]

New England Journal of Medicine, 1992
We previously described two members of a family affected by an apparently genetically determined fatal disease characterized clinically by progressive insomnia, dysautonomia, and motor signs and characterized pathologically by severe atrophy of the anterior ventral and mediodorsal thalamic nuclei.
Rossella Medori, Hans-Juergen Tritschler, Andréa LeBlanc, Federico Villare, Valeria Manetto, Hsiao Ying Chen, Run Xue, Suzanne Leal, Pasquale Montagna, Pietro Cortelli, Paolo Tinuper, Patrizia Avoni, Mirella Mochi, Agostino Baruzzi, Jean Jaques Hauw, Jurg Ott, Elio Lugaresi, Lucila Autilio-Gambetti, Pierluigi Gambetti   +18 more
openaire   +2 more sources

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

Novel Insertion/Deletion Polymorphisms and Genetic Studies of the Shadow of Prion Protein (SPRN) in Raccoon Dogs

Animals
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. One of the candidate genes involved in prion diseases is the shadow of the prion protein (SPRN) gene. Raccoon dogs, a canid,
Da-In Choi, Mohammed Zayed, Eun-Jee Na, Jae-Ku Oem, Byung-Hoon Jeong   +4 more
doaj   +1 more source