نتایج جستجو برای: ژن prnp

تعداد نتایج: 16699  

2017
Guohong Jiang Changming Wang Jun Zhang Haijun Liu

The aim of the study was to examine the mediation of insulin growth factor-1 (IGF-1) in Alzheimer's disease (AD), as well as the underlying mechanism of the PRNP genetic expression and PI3K/Akt signaling pathway. The Aβ25-35-incubated rat adrenal pheochromocytoma cell (PC12) in vitro was established, constituting the AD model. Different doses (0, 20, 40 and 80 ng/ml) of IGF-1 were used in PC12 ...

2017
Hui Zhao Siqi Wang Lixia Guo Yanli Du Linlin Liu Tengfei Ma Newton O. Otecko Canpeng Li Yaping Zhang

Bovine spongiform encephalopathy, a member of transmissible spongiform encephalopathies, has not been reported in buffaloes, Bubalus bubalis. Prion protein (PrP), encoded by the prion protein gene (PRNP), is fundamental in the pathogenesis of transmissible spongiform encephalopathies. We previously showed that buffaloes express more PrP proteins but lower PRNP mRNA than cattle in several pivota...

2014
Byung-Hoon Jeong Yong-Sun Kim

Human prion diseases are fatal neurodegenerative disorders that are characterized by spongiform changes, astrogliosis, and the accumulation of an abnormal prion protein (PrP(Sc)). Approximately 10%-15% of human prion diseases are familial variants that are caused by pathogenic mutations in the prion protein gene (PRNP). Point mutations or the insertions of one or more copies of a 24 bp repeat a...

Journal: :Folia neuropathologica 2012
Jolanta Bratosiewicz-Wąsik Joanna Smoleń-Dzirba Cezary Watała Annemieke J Rozemuller Casper Jansen Wim Spliet Gerard H Jansen Tomasz J Wąsik Paweł P Liberski

The prion protein (PrP) plays a central role in the pathogenesis of Creutzfeldt-Jakob disease and other transmissible spongiform encephalopathies (TSEs). Mutations in the coding region of the prion protein (PRNP) gene are linked to inherited forms of TSEs whereas aetiology of sporadic CJD (sCJD) remains obscure. It remains unclear whether the primary DNA sequence at non-coding region of PRNP ge...

2016
José A. del Río Rosalina Gavín

Since its discovery the cellular prion protein (encoded by the Prnp gene) has been associated with a large number of functions. The proposed functions rank from basic cellular processes such as cell cycle and survival to neural functions such as behavior and neuroprotection, following a pattern similar to that of Moore's law for electronics. In addition, particular interest is increasing in the...

Journal: :Journal of animal science 2006
B J Isler B A Freking R M Thallman M P Heaton K A Leymaster

There is concern about potential antagonistic correlated responses due to intensive selection for scrapie-resistant haplotypes of the prion (PRNP) gene in sheep. The objective of the present research was to test for associations of PRNP haplotypes for codons 136, 154, and 171 with growth, carcass, and meat quality traits in an F2 Dorset x Romanov population (n = 415) segregating the 2 callipyge...

Journal: :Human molecular genetics 2012
Simon Mead James Uphill John Beck Mark Poulter Tracy Campbell Jessica Lowe Gary Adamson Holger Hummerich Norman Klopp Ina-Maria Rückert H-Erich Wichmann Dhoyazan Azazi Vincent Plagnol Wandagi H Pako Jerome Whitfield Michael P Alpers John Whittaker David J Balding Inga Zerr Hans Kretzschmar John Collinge

Prion diseases are fatal neurodegenerative diseases of humans and animals caused by the misfolding and aggregation of prion protein (PrP). Mammalian prion diseases are under strong genetic control but few risk factors are known aside from the PrP gene locus (PRNP). No genome-wide association study (GWAS) has been done aside from a small sample of variant Creutzfeldt-Jakob disease (CJD). We cond...

2015
Brenda M. Murdoch Gordon K. Murdoch

Bovine spongiform encephalopathy (BSE) is a prion disease that is invariably fatal in cattle and has been implicated as a significant human health risk. As a transmissible disease of livestock, it has impacted food safety, production practices, global trade, and profitability. Genetic polymorphisms that alter the prion protein in humans and sheep are associated with transmissible spongiform enc...

Journal: :The Journal of biological chemistry 2015
Mario Nuvolone Adriano Aguzzi

Beckman et al. (1) reported altered monoaminergic systems and depressive-like behaviors in a line of mice lacking the cellular prion protein PrP (congenic B10.129-Prnp mice). These mice were derived from gene-targeted 129/Ola embryonic stem cells and were subsequently crossed to C57BL/10SnJ mice. Consequently, the genomic region of B10.129-Prnp mice flanking the targeted Prnp locus contains a s...

Journal: :Animal genetics 2013
H Meydan M M Ozkan M A Yildiz W Goldmann

The aim of this study was to identify the PRNP polymorphisms outside the standard codons 136, 154 and 171 in 1110 sheep with no clinical sign of scrapie from all 18 Turkish native sheep breeds and compare our results with published data on ovine PRNP polymorphism from other regions of the world. Among the 22 amino acid polymorphisms and three silent mutations, 10 were novel for ovine PRNP: p.Gl...

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