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221por Diaz-Espinoza, Rodrigo, Morales, Rodrigo, Concha-Marambio, Luis, Moreno-Gonzalez, Ines, Moda, Fabio, Soto, Claudio“…Transmissible Spongiform Encephalopathies (TSEs) are fatal neurological disorders caused by prions, which are composed of a misfolded protein (PrP(Sc)) that self-propagates in the brain of infected individuals by converting the normal prion protein (PrP(C)) into the pathological isoform. …”
Publicado 2017
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222por Zheng, Zhen, Zhang, Meilan, Wang, Yongheng, Ma, Rongsheng, Guo, Chenyun, Feng, Liubin, Wu, Jihui, Yao, Hongwei, Lin, Donghai“…Prion diseases are caused by the propagation of misfolded cellular prion proteins (PrPs). …”
Publicado 2018
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223por Ashok, Ajay, Karmakar, Shilpita, Chandel, Rajeev, Ravikumar, Ranjana, Dalai, Stuti, Kong, Qingzhong, Singh, Neena“…Here, we explored whether the ciliary body (CB), the source of aqueous humor, transports iron, and if the prion protein (PrP(C)) facilitates this process as in the outer retina. …”
Publicado 2018
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224por Igel-Egalon, Angélique, Bohl, Jan, Moudjou, Mohammed, Herzog, Laetitia, Reine, Fabienne, Rezaei, Human, Béringue, Vincent“…Prions are proteinaceous infectious agents responsible for a range of neurodegenerative diseases in animals and humans. …”
Publicado 2019
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225por Pan, Chenhua, Yang, Junwei, Zhang, Xiangyi, Chen, Ying, Wei, Shunxiong, Yu, Guohua, Pan, Yi-Hsuan, Ma, Jiyan, Yuan, Chonggang“…The prion hypothesis postulates that PrP(Sc), the pathogenic conformer of host-encoded prion protein (PrP), is the unconventional proteinaceous infectious agent called prion. …”
Publicado 2020
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226“…Prion diseases are a group of neurodegenerative diseases endemic in humans and several ruminants caused by the misfolding of native prion protein (PrP) into pathological conformations. …”
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227por Uchiyama, Keiji, Miyata, Hironori, Yamaguchi, Yoshitaka, Imamura, Morikazu, Okazaki, Mariya, Pasiana, Agriani Dini, Chida, Junji, Hara, Hideyuki, Atarashi, Ryuichiro, Watanabe, Hitomi, Kondoh, Gen, Sakaguchi, Suehiro“…Conformational conversion of the cellular prion protein, PrP(C), into the abnormally folded isoform, PrP(Sc), is a key pathogenic event in prion diseases. …”
Publicado 2020
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228por Minikel, Eric Vallabh, Zhao, Hien T, Le, Jason, O’Moore, Jill, Pitstick, Rose, Graffam, Samantha, Carlson, George A, Kavanaugh, Michael P, Kriz, Jasna, Kim, Jae Beom, Ma, Jiyan, Wille, Holger, Aiken, Judd, McKenzie, Deborah, Doh-ura, Katsumi, Beck, Matthew, O’Keefe, Rhonda, Stathopoulos, Jacquelyn, Caron, Tyler, Schreiber, Stuart L, Carroll, Jeffrey B, Kordasiewicz, Holly B, Cabin, Deborah E, Vallabh, Sonia M“…Lowering of prion protein (PrP) expression in the brain is a genetically validated therapeutic hypothesis in prion disease. …”
Publicado 2020
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229por Hara, Hideyuki, Chida, Junji, Uchiyama, Keiji, Pasiana, Agriani Dini, Takahashi, Etsuhisa, Kido, Hiroshi, Sakaguchi, Suehiro“…Misfolding of the cellular prion protein, PrP(C), into the amyloidogenic isoform, PrP(Sc), which forms infectious protein aggregates, the so-called prions, is a key pathogenic event in prion diseases. …”
Publicado 2021
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230por Hernaiz, Adelaida, Toivonen, Janne Markus, Bolea, Rosa, Martín-Burriel, Inmaculada“…Prion diseases are transmissible spongiform encephalopathies (TSEs) caused by a conformational conversion of the native cellular prion protein (PrP(C)) to an abnormal, infectious isoform called PrP(Sc). …”
Publicado 2022
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231por Goodwill, Vanessa S., Dryden, Ian, Choi, Jihee, De Lillo, Chiara, Soldau, Katrin, Llibre-Guerra, Jorge, Sanchez, Henry, Sigurdson, Christina J., Lin, Jonathan H.“…Sporadic Creutzfeldt-Jakob disease (sCJD) is the most commonly diagnosed human prion disease caused by the abnormal misfolding of the ‘cellular’ prion protein (PrP(C)) into the transmissible ‘scrapie-type’ prion form (PrP(Sc)). …”
Publicado 2022
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233“…The number of yeast prions and prion-like proteins described since 1994 has grown from two to nearly twenty. …”
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234“…A report on a Joint Cold Spring Harbor Laboratory/Wellcome Trust Conference on 'Prion Biology', Hinxton, UK, 7-11 September 2005.…”
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235por Clawson, Michael L, Heaton, Michael P, Keele, John W, Smith, Timothy PL, Harhay, Gregory P, Laegreid, William W“…BACKGROUND: Bovine spongiform encephalopathy (BSE) is a fatal neurological disorder characterized by abnormal deposits of a protease-resistant isoform of the prion protein. Characterizing linkage disequilibrium (LD) and haplotype networks within the bovine prion gene (PRNP) is important for 1) testing rare or common PRNP variation for an association with BSE and 2) interpreting any association of PRNP alleles with BSE susceptibility. …”
Publicado 2006
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236“…We made use of the present availability of vertebrate genomic sequences, and we have conducted the most comprehensive comparative genomic analysis of the prion protein gene PRNP and its homologues, shadow of prion protein gene SPRN and doppel gene PRND, and prion testis-specific gene PRNT so far. …”
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237por Genovesi, Sacha, Leita, Liviana, Sequi, Paolo, Andrighetto, Igino, Sorgato, M. Catia, Bertoli, Alessandro“…Scrapie and chronic wasting disease are contagious prion diseases affecting sheep and cervids, respectively. …”
Publicado 2007
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239“…Prion diseases are caused by proteinaceous pathogens termed prions. …”
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240“…Capacitor systems, which allow the accumulation of cryptic genetic variation and release it under stressful conditions, might provide such a mechanism. In yeast, the prion [PSI(+)] exposes a large array of previously hidden genetic variation, and the phenotypes it thereby produces are advantageous roughly 25% of the time. …”
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