نتایج جستجو برای: ژن prnp

تعداد نتایج: 16699  

2015
Lisa Gasperini Elisa Meneghetti Beatrice Pastore Federico Benetti Giuseppe Legname

AIMS Several neurodegenerative disorders show alterations in glutamatergic synapses and increased susceptibility to excitotoxicity. Mounting evidence suggests a central role for the cellular prion protein (PrP(C)) in neuroprotection. Therefore, the loss of PrP(C) function occurring in prion disorders may contribute to the disease progression and neurodegeneration. Indeed, PrP(C) modulates N-met...

Journal: :Animal genetics 2010
P Mesquita M Batista M R Marques I C Santos J Pimenta M Silva Pereira I Carolino F Santos Silva M C Oliveira Sousa L T Gama C M Fontes A E M Horta J A M Prates R M Pereira

The establishment of an association between prion protein gene (PRNP) polymorphisms and scrapie susceptibility in sheep has enabled the development of breeding programmes to increase scrapie resistance in the European Union. Intense selection for PRNP genotype may lead to correlated selection for genes linked to PRNP. We intended to investigate if any association exists between genetic variatio...

Journal: :The Journal of heredity 2007
Christopher M Seabury Clare A Gill Joe W Templeton Joseph B Dyar James N Derr David L Adelson Elaine Owens Donald S Davis Duane C Kraemer James E Womack

Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) affecting deer (Odocoileus spp.), moose (Alces alces), and Rocky Mountain elk (Cervus elaphus nelsoni). Leucine homozygosity at elk PRNP codon 132 has been associated with reduced CWD susceptibility. However, naturally acquired CWD has been detected in elk possessing the 132 Leu/Leu genotype. Recent human and bovin...

2014
Akikazu Sakudo Takashi Onodera

Elucidation of prion protein (PrP) functions is crucial to fully understand prion diseases. A major approach to studying PrP functions is the use of PrP gene-knockout (Prnp (-/-)) mice. So far, six types of Prnp (-/-) mice have been generated, demonstrating the promiscuous functions of PrP. Recently, other PrP family members, such as Doppel and Shadoo, have been found. However, information obta...

2017
Marc-André Déry Andréa C. LeBlanc

The cellular prion protein (PrP) is essential for transmissible prion diseases, but its exact physiological function remains unclear. Better understanding the regulation of the human prion protein gene (PRNP) expression can provide insight into this elusive function. Spliced XBP1 (sXBP1) was recently shown to mediate endoplasmic reticulum (ER) stress-induced PRNP expression. In this manuscript,...

2013
Ting Liu Wenjing Yi Boya Feng Zheng Zhou Gengfu Xiao

The conformational conversion of the cellular prion protein (PrP(C)) into its β-sheet-rich scrapie isoform (PrP(Sc)) causes fatal prion diseases, which are also called transmissible spongiform encephalopathies (TSEs). Recent studies suggest that the expression of PrP(C) by the PRNP gene is crucial for the development of TSEs. Therefore, the identification of the exogenous and endogenous stimula...

Journal: :International journal of molecular medicine 2007
Chi-Kyeong Kim Akikazu Sakudo Yojiro Taniuchi Kazuto Shigematsu Chung-Boo Kang Keiichi Saeki Yoshitsugu Matsumoto Suehiro Sakaguchi Shigeyoshi Itohara Takashi Onodera

Several lines of prion protein gene (Prnp)-knockout mice such as ZrchI, ZrchII, Npu, Ngsk and Rcm0 have been generated. Of these, ZrchII, Ngsk and Rcm0 exhibit late-onset ataxia due to ectopic expression of Doppel (Dpl); a result of damage to the splicing acceptor of Prnp exon 3. Recently, we developed another line of Prnp-/- mice (Rikn), which was generated by gene targeting with more nucleoti...

2013
Yasushi Kishimoto Moritoshi Hirono Ryuichiro Atarashi Suehiro Sakaguchi Tohru Yoshioka Shigeru Katamine Yutaka Kirino

Mice lacking the prion protein (PrP(C)) gene (Prnp), Ngsk Prnp (0/0) mice, show late-onset cerebellar Purkinje cell (PC) degeneration because of ectopic overexpression of PrP(C)-like protein (PrPLP/Dpl). Because PrP(C) is highly expressed in cerebellar neurons (including PCs and granule cells), it may be involved in cerebellar synaptic function and cerebellar cognitive function. However, no stu...

2015
Malin R. Reiten Maren K. Bakkebø Hege Brun-Hansen Anna M. Lewandowska-Sabat Ingrid Olsaker Michael A. Tranulis Arild Espenes Preben Boysen

The physiological role of the cellular prion protein (PrP(C)) is incompletely understood. The expression of PrP(C) in hematopoietic stem cells and immune cells suggests a role in the development of these cells, and in PrP(C) knockout animals altered immune cell proliferation and phagocytic function have been observed. Recently, a spontaneous nonsense mutation at codon 32 in the PRNP gene in goa...

Journal: :Science 2003
Simon Mead Michael P H Stumpf Jerome Whitfield Jonathan A Beck Mark Poulter Tracy Campbell James B Uphill David Goldstein Michael Alpers Elizabeth M C Fisher John Collinge

Kuru is an acquired prion disease largely restricted to the Fore linguistic group of the Papua New Guinea Highlands, which was transmitted during endocannibalistic feasts. Heterozygosity for a common polymorphism in the human prion protein gene (PRNP) confers relative resistance to prion diseases. Elderly survivors of the kuru epidemic, who had multiple exposures at mortuary feasts, are, in mar...

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