نتایج جستجو برای: prnp

تعداد نتایج: 934  

2014
Walker S. Jackson Clemens Krost Andrew W. Borkowski Lech Kaczmarczyk

Prion diseases induce neurodegeneration in specific brain areas for undetermined reasons. A thorough understanding of the localization of the disease-causing molecule, the prion protein (PrP), could inform on this issue but previous studies have generated conflicting conclusions. One of the more intriguing disagreements is whether PrP is synthesized by astrocytes. We developed a knock-in report...

Journal: :Journal of Applied Ecology 2023

Many pathogens can infect several host species, which complicates the management of wildlife diseases. Even for generalist pathogens, hosts are not equally competent, and variable niche overlap between leads to different exposure levels within when compared with that hosts. Hence, processes determining spillover risk subsequent transmission dynamics species differ. Chronic wasting disease (CWD)...

2014
Abigail B. Diack Diane L. Ritchie Alexander H. Peden Deborah Brown Aileen Boyle Laura Morabito David Maclennan Paul Burgoyne Casper Jansen Richard S. Knight Pedro Piccardo James W. Ironside Jean C. Manson

Variably protease-sensitive prionopathy (VPSPr) can occur in persons of all codon 129 genotypes in the human prion protein gene (PRNP) and is characterized by a unique biochemical profile when compared with other human prion diseases. We investigated transmission properties of VPSPr by inoculating transgenic mice expressing human PRNP with brain tissue from 2 persons with the valine-homozygous ...

Journal: :The Veterinary record 2016
W Goldmann E Marier P Stewart T Konold S Street J Langeveld O Windl A Ortiz-Pelaez

Scrapie in goats is a transmissible, fatal prion disease, which is endemic in the British goat population. The recent success in defining caprine PRNP gene variants that provide resistance to experimental and natural classical scrapie has prompted the authors to conduct a survey of PRNP genotypes in 10 goat breeds and 52 herds to find goats with the resistant K222 allele. They report here the f...

Journal: :Essays in biochemistry 1998
A Aguzzi M A Klein C Musahl A J Raeber T Blättler I Hegyi R Frigg S Brandner

For the study of prion neurotoxicity, we used neural-grafting techniques: mice devoid of the normal host prion protein (Prnp% mice) received a neural graft and were intracerebrally infected with mouse prions. The growth and differentiation properties of neural grafts were defined. Growth of embryonic neuroectodermal tissue was optimal at gestational days 12.5-13.5. The blood-brain barrier is re...

Journal: :BMB reports 2009
Yang Yang Lan Chen Hua-Zhen Pan Yi Kou Cai-Min Xu

We investigate the correlation between the glycosylation modified prion proteins and apoptosis. The wild-type PRNP gene and four PRNP gene glycosylated mutants were transiently expressed in HeLa cells. The effect of apoptosis induced by PrP mutants was confirmed by MTT assay, Hochest staining, Annexin-V staining and PI staining. ROS test detected ROS generation within the cells. The mitochondri...

Journal: :Journal of neurology, neurosurgery, and psychiatry 2010
C Jansen M W Head W A van Gool F Baas H Yull J W Ironside A J M Rozemuller

An atypical case of prion disease is described in a 54-year-old Dutch man, homozygous for valine at codon 129 of the prion protein gene (PRNP). The clinical phenotype was characterised by progressive dementia, spastic paraplegia and sensorimotor polyneuropathy. The disease duration was 20 months. Genetic analysis of PRNP did not reveal any abnormalities. Neuropathologically, only mild spongifor...

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