A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83)
Excerpt | Reference |
"The prion diseases are neurodegenerative conditions, transmissible by inoculation, and in some cases inherited as an autosomal dominant disorder." | ( Clarke, AR; Collinge, J; Jefferys, JG; Palmer, MS; Sidle, KC; Smith, CJ; Whittington, MA, 1994) |
"Transmissible spongiform encephalopathies are a unique group of brain diseases of different animal species and man that can be transmitted between individuals by inoculation or ingestion of diseased nervous system tissues." | ( Fritzemeier, J; Moennig, V, 1997) |
"Prion diseases are typically initiated by infection of peripheral sites, as in the case of bovine spongiform encephalopathy, new variant Creutzfeldt-Jakob disease, kuru and most cases of iatrogenic Creutzfeldt-Jakob disease." | ( Aguzzi, A; Flechsig, E; Frigg, R; Hegyi, I; Klein, MA; Raeber, AJ; Weissmann, C; Zinkernagel, RM, 1998) |
"Prion diseases are neurodegenerative disorders associated with a conformational change in the normal cellular isoform of the prion protein, PrP(C), to an abnormal scrapie isoform, PrP(SC)." | ( Good, TA; Rymer, DL, 2000) |
"Transmissible spongiform encephalopathies are associated with accumulation of PrP(Sc), a conformer of a cellular protein called PrP(C)." | ( Aguzzi, A; Fischer, MB; Parizek, P; Roeckl, C; Schwarz, HP, 2000) |
"Transmissible spongiform encephalopathies are believed to be caused by an infectious form of the prion protein, designated PrP(Sc)." | ( Jansen, VA; Masel, J, 2001) |
"One aspect of prion disease is the conversion of functional prion protein into an aggregated amyloid." | ( Brown, DR, 2001) |
"Prion diseases are fatal and transmissible neurodegenerative disorders linked to an aberrant conformation of the cellular prion protein (PrP(c))." | ( Gilch, S; Groschup, MH; Lucassen, R; Muranyi, W; Nunziante, M; Riesner, D; Schätzl, HM; Spielhaupter, C; Tatzelt, J; Winklhofer, KF, 2001) |
"Transmissible spongiform encephalopathies are characterised by the transformation of the normal cellular prion protein (PrP(C)) into an abnormal isoform (PrP(TSE))." | ( Beyreuther, K; Cappai, R; Collins, SJ; Jobling, MF; Maher, F; Masters, CL; Needham, BE; Stewart, LR; Thyer, J; White, AR, 2001) |
"Prion diseases are not only genetic or sporadic neurodegenerative disorders, but more important, they are transmissible diseases." | ( Brown, DR; Sy, MS; Wong, BS, 2001) |
"Prion diseases are characterized by accumulation of protease resistant isoforms of prion protein (termed PrP(SC)), glial activation and neurodegeneration." | ( Boshuizen, RS; Eikelenboom, P; Hoozemans, JJ; Janssen, I; Langeveld, JP; Veerhuis, R, 2002) |
"Prion diseases are neurodegenerative pathologies characterized by the accumulation in the brain of a protease-resistant form of the prion protein (PrP(c)), named PrP(Sc)." | ( Arena, S; Benatti, U; Corsaro, A; Damonte, G; Florio, T; Millo, E; Schettini, G; Tagliavini, F; Thellung, S; Villa, V, 2002) |
"Prion diseases or transmissible spongiform encephalopathies are neurodegenerative disorders that are genetic, sporadic, or infectious." | ( Bose, S; Gu, Y; Kalepu, S; Mishra, RS; Singh, N; Verghese, S, 2002) |
"Prion diseases are associated with the conversion of the normal prion protein, PrP(C), to the infectious disease form PrP(Sc)." | ( Brown, DR; Daniels, M; Ellis, V; Misra, R, 2002) |
"Transmissible spongiform encephalopathies are diseases of animals and humans that are also termed prion diseases." | ( Brown, DR, 2002) |
"Prion diseases are characterized by an accumulation of PrP(Sc), a misfolded isoform of the normal cellular prion protein, PrP(C)." | ( Cohen, FE; Deady, LW; Fafarman, AT; Hong, SB; May, BC; Prusiner, SB; Rogers, M, 2003) |
"Prion diseases are fatal, chronic neurodegenerative diseases of mammals, characterized by amyloid deposition, astrogliosis, microglial activation, tissue vacuolation and neuronal loss." | ( Boche, D; Cunningham, C; Deacon, R; Diniz, CP; Perry, VH; Rawlins, JN; Scott, H; Waters, S; Wells, H, 2003) |
"Prion diseases or transmissible spongiform encephalopathies are a group of closely related transmissible neurodegenerative conditions of humans and animals, all of which are incurable." | ( Elliott, CL; Tabrizi, SJ; Weissmann, C, 2003) |
"Prion diseases are neurodegenerative conditions." | ( Brown, DR; Daniels, M; Sassoon, J, 2004) |
"Central to Prion diseases is the normal endogenous Prion protein, PrPC." | ( Comerford, EJ; Deignan, ME; McMahon, HE; Prior, M; Stuart, LE, 2004) |
"Prion diseases are caused by the accumulation of an aberrantly folded isoform of the prion protein, designated PrPSc." | ( Baldwin, M; Guglielmo, BJ; Huang, Y; Legname, G; Lessard, P; Lin, ET; Prusiner, SB; Ryou, C; Yung, L, 2004) |
"Inherited prion diseases are linked to mutations in the prion protein (PrP) gene, which favor conversion of PrP into a conformationally altered, pathogenic isoform." | ( Chiesa, R; Dossena, S; Fioriti, L; Forloni, G; Harris, DA; Stewart, LR; Stewart, RS, 2005) |
"Prion diseases are transmissible neurodegenerative disorders characterized by extensive neuronal apoptosis and accumulation of misfolded prion protein (PrP(SC))." | ( Carboni, S; Castilla, J; Hetz, C; Maundrell, K; Russelakis-Carneiro, M; Soto, C; Vial-Knecht, E; Wälchli, S, 2005) |
"Prion diseases are characterized by the accumulation of diffuse and aggregated plaques of protease-resistant prion protein (PrP) in the brains of affected individuals and animals." | ( Budka, H; Castellani, RJ; Cras, P; Gambetti, P; Ghetti, B; Guentchev, M; Ironside, JW; Kim, YS; Lee, HG; Moreira, PI; Nunomura, A; Perry, G; Petersen, RB; Siedlak, SL; Smith, MA; Tagliavini, F, 2005) |
"Prion diseases are characteristically accompanied by marked astrocytic activation, which is initiated relatively early in the disease process." | ( Gray, BC; O'Connor, VM; Perry, VH; Skipp, P, 2006) |
"Inherited prion diseases are caused by mutations in the gene which codes for prion protein (PrP), leading to proliferation of abnormal PrP isomers in the brain and neurodegeneration; they include Gerstmann-Sträussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and familial Creutzfeldt-Jakob disease (fCJD)." | ( Collinge, J; Cordery, RJ; Godbolt, A; MacManus, DG; Rossor, MN; Waldman, AD, 2006) |
"Prion diseases are fatal neurodegenerative disorders associated with the conversion of the cellular prion protein (PrPC) into a pathologic isoform." | ( Chacón, MA; Inestrosa, NC; Toledo, EM; Varela-Nallar, L, 2006) |
"Prion diseases are transmissible, invariably fatal, neurodegenerative diseases which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy and scrapie in animals." | ( Collinge, J; Trevitt, CR, 2006) |
"Prion diseases are fatal neurodegenerative disorders associated with conformational conversion of the cellular prion protein (PrP(C)) into an isoform designated PrP(Sc)." | ( González, A; Inestrosa, NC; Varela-Nallar, L, 2006) |
"Prion diseases are fatal and at present there are neither cures nor palliative therapies known/available, which delay disease onset or progression." | ( Baier, M; Bamme, T; Gültner, S; Lütjohann, D; Mok, SW; Riemer, C; Thelen, KM, 2006) |
"Transmissible spongiform encephalopathies are a group of fatal, neurodegenerative diseases commonly known as prion diseases." | ( Collin, R; Harris, K; Keevil, CW; Lipscomb, IP; Pinchin, HE, 2006) |
"1." | ( Nikles, D; Vana, K; Weiss, S; Zuber, C, 2007) |
"Propagation of transmissible spongiform encephalopathies is associated with the conversion of normal prion protein, PrP(C), into a misfolded, oligomeric form, PrP(Sc)." | ( Lu, X; Surewicz, WK; Wintrode, PL, 2007) |
"Prion diseases are fatal chronic neurodegenerative diseases." | ( Anthony, DC; Blamire, AM; Broom, KA; Griffin, JL; Lowe, JP; Perry, VH; Scott, H; Sibson, NR; Styles, P, 2007) |
"Prion diseases are fatal neurodegenerative infectious disorders for which no therapeutic or prophylactic regimens exist." | ( Ertmer, A; Gilch, S; Nunziante, M; Schätzl, HM, 2007) |
"Transmissible spongiform encephalopathies are a group of neurological disorders associated with the deposition of PrP(Sc), an abnormal form of the cellular prion protein PrP(c)." | ( Arnold, GJ; Fröhlich, T; Knackmuss, S; Kretzschmar, HA; Little, M; Mitteregger, G; Pace, C; Reusch, U; Rey, C; Röttgen, P; Weiss, S; Zuber, C, 2008) |
"Prion diseases are fatal neurodegenerative disorders." | ( Brown, DR; Gilbert, IH; Lekishvili, T; Loeschner, C; Prelli, F; Sellarajah, S; Webb, S; Wisniewski, T, 2007) |
"Prion diseases, or transmissible spongiform encephalopathies, are a heterogeneous group of fatal neurodegenerative disorders, characterized by deposition of the protease-resistant prion protein, astrocytosis, and spongiform degeneration." | ( Minghetti, L; Pocchiari, M, 2007) |
"Prion diseases are fatal neurodegenerative disorders that are caused by the conversion of a normal host-encoded protein, PrP(C), to an abnormal, disease-causing form, PrP(Sc)." | ( Lehmann, S; McMahon, HE; Molloy, B; Prior, M; Sy, MS, 2007) |
"Prion diseases are fatal neurodegenerative diseases that are characterized by the conformational conversion of the normal, mainly alpha-helical cellular prion protein (PrP) into the abnormal beta-sheet-rich infectious isoform (PrP(Sc))." | ( Hammerschmidt, F; Heit, A; Hess, S; Jennen, L; Kaiser-Schulz, G; Quintanilla-Martinez, L; Rezaei, H; Schätzl, HM; Wagner, H, 2007) |
"Prion diseases are fatal neurodegenerative disorders." | ( Brown, DR; Uppington, KM, 2008) |
"Prion diseases are fatal and at present there are neither cures nor therapies available to delay disease onset or progression in humans." | ( Baier, M; Burwinkel, M; Gültner, S; Heise, I; Holtkamp, N; Mok, SWF; Riemer, C; Schwarz, A, 2008) |
"Prion disease or transmissible spongiform encephalopathies are neurodegenerative disorders of humans and other mammals." | ( Brown, DR; Hesketh, S; Knight, R; Sassoon, J, 2008) |
"Prion diseases are a group of fatal neurodegenerative diseases affecting humans and animals." | ( Avrahami, D; Friedman-Levi, Y; Gabizon, R; Mayer-Sonnenfeld, T, 2008) |
"Prion diseases are fatal neurodegenerative diseases that can induce endocrinopathies." | ( Bailey, JD; Berardinelli, JG; Bessen, RA; Rocke, TE, 2008) |
"A hallmark in prion diseases is the conformational transition of the cellular prion protein (PrP(C)) into a pathogenic conformation, designated scrapie prion protein (PrP(Sc)), which is the essential constituent of infectious prions." | ( Baier, M; Becker, CF; Brumm, L; Engelhard, M; Gazit, E; Levy, M; Miesbauer, M; Oesterhelt, D; Olschewski, D; Rambold, AS; Riemer, C; Seidel, R; Smale, L; Tatzelt, J; Winklhofer, KF, 2008) |
"Prion diseases are fatal neurodegenerative and infectious disorders for which no therapeutic or prophylactic regimens exist." | ( Aguib, Y; Baier, M; Ertmer, A; Gilch, S; Heiseke, A; Riemer, C; Schätzl, HM, 2009) |
"Prion diseases are associated with the misfolding of the host-encoded cellular prion protein (PrP(C)) into a disease associated form (PrP(Sc))." | ( Cappai, R; Coleman, BM; Han, S; Hatters, DM; Hill, AF; Nisbet, RM, 2009) |
"The main feature of prion diseases is the accumulation of infectious proteins (PrP(Sc))." | ( Groschup, MH; Karch, H; Kuczius, T, 2009) |
"Prion diseases are a group of fatal neurodegenerative disorders that manifest as infectious, sporadic, or familial and are all associated with the misfolding of the prion protein (PrP)." | ( Pinheiro, TJ; Robinson, PJ, 2009) |
"Prion diseases are fatal infectious neurodegenerative disorders; examples include the Creutzfeldt-Jakob disease affecting humans and bovine spongiform encephalopathy in cattle." | ( Sakaguchi, S, 2009) |
"Prion diseases are rare, rapidly progressive, fatal neurodegenerative illnesses caused by an abnormal isoform of the native prion protein." | ( Appleby, BS, 2009) |
"Prion diseases are caused by the conversion of a cellular protein (PrP(C)) into a misfolded, aggregated isoform (PrP(Res))." | ( Côté, S; Roostaee, A; Roucou, X, 2009) |
"Prion diseases are neurodegenerative diseases associated with the accumulation of a pathogenic isoform of the host-encoded prion protein." | ( Bach, C; Beckers, J; Brodesser, S; Facius, A; Gilch, S; Greenwood, AD; Hajj, GN; Horsch, M; Leib-Mösch, C; Rost, R; Sandhoff, K; Schädler, S; Schätzl, HM; Vorberg, I, 2009) |
"Prion diseases are neurodegenerative diseases affecting humans and animals in which the infectious agent or prion is PrP(res), a protease-resistant conformer of the cell protein PrP." | ( Bareggi, SR; Bondiolotti, G; Formentin, E; Lucchini, B; Poli, G; Ponti, W; Puricelli, M; Rossoni, G, 2010) |
"Prion diseases are considered to be transmissible." | ( Brown, DR; Davies, P, 2009) |
"Prion diseases are fatal, untreatable neurodegenerative diseases caused by the accumulation of the misfolded, infectious isoform of the prion protein (PrP), termed PrP(Sc)." | ( Ghaemmaghami, S; May, BC; Prusiner, SB; Renslo, AR, 2010) |
"Prion diseases are fatal neurodegenerative diseases resulting from misfolding of normal cellular prion (PrP(C)) into an abnormal form of scrapie prion (PrP(Sc))." | ( Anantharam, V; Choi, CJ; Kanthasamy, A; Kanthasamy, AG; Martin, DP; Nicholson, EM; Richt, JA, 2010) |
"Prion diseases are a group of etiologically heterogeneous neurodegenerative disorders." | ( Brajusković, G; Cadez, I; Dimitrijević, R; Dobricić, V; Keckarević, D; Keckarević-Marković, M; Kecmanović, M; Romac, S; Savić-Pavićević, D, 2010) |
"Prion diseases are fatal neurodegenerative disorders characterized by a long pre-symptomatic phase followed by rapid and progressive clinical phase." | ( Gonzalez-Romero, D; Green, K; Morales-Scheihing, D; Mukherjee, A; Soto, C; Taglialatela, G, 2010) |
"Prion diseases are transmissible between different species, however, with a variable species barrier." | ( Birkmann, E; Luers, L; Nagel-Steger, L; Panza, G; Riesner, D; Stöhr, J; Weiss, J; Willbold, D, 2010) |
"Prion diseases are neurodegenerative disorders characterized by deposition of the pathological prion protein (PrPsc) within the brain of affected humans and animals." | ( Chabry, J; Di Marzo, V; Ménard, B; Petrosino, S; Zsürger, N, 2011) |
"Prion diseases are characterized by protein conformational conversion from a normal cellular form (PrP(C)) to an abnormal scrapie isoform (PrP(Sc))." | ( Du, W; Feng, L; Huang, C; Li, Y; Wang, X; Wang, Y; Zhang, B, 2011) |
"Prion disease is a classical neurodegenerative disorder resulting from the misfolding of endogenously expressed normal cellular prion protein (PrP(C))." | ( Anantharam, V; Houk, R; Jin, H; Kanthasamy, A; Kanthasamy, AG; Martin, DP; Witte, T, 2011) |
"Human prion diseases are heterogeneous but invariably fatal neurodegenerative disorders with no known effective therapy." | ( Collinge, J; Darbyshire, JH; Gopalakrishnan, GS; Hudson, F; Kennedy, A; MacKay, A; Mead, S; Ranopa, M; Rudge, P; Thompson, AG; Walker, AS; Wroe, S, 2011) |
"Prion diseases are a group of incurable transmissible neurodegenerative disorders." | ( Bi, H; Shimoji, M; Wang, GX; Wang, Y; Wong, J; Xiao, X; Yuan, J; Zhou, X; Zou, WQ, 2011) |
"Prion diseases are diagnosed by the detection of their proteinase K-resistant prion protein fragment (PrP(Sc))." | ( Benestad, SL; Brenig, B; Breyer, J; Graham, C; Richt, JA; Schulz-Schaeffer, WJ; Wemheuer, WM; Wrede, A, 2012) |
"Prion diseases are a class of fatal neurodegenerative disorders characterized by brain spongiosis, synaptic degeneration, microglia and astrocytes activation, neuronal loss and altered redox control." | ( Arcovito, A; Benetti, F; Chillemi, G; D'Angelo, P; Della Longa, S; Giachin, G; Legname, G; Mancini, G; Zitolo, A, 2012) |
"Prion diseases are fatal neurodegenerative disorders associated with the polymerization of the cellular form of prion protein (PrP(C)) into an amyloidogenic β-sheet infectious form (PrP(Sc))." | ( Cortez, LM; Kumar, J; Renault, L; Sim, VL; Young, HS, 2013) |
"Prion diseases are fatal neurodegenerative disorders involving the abnormal folding of a native cellular protein, named PrP(C), to a malconformed aggregation-prone state, enriched in beta sheet secondary structure, denoted PrP(Sc)." | ( Browman, D; Caputo, A; Chamoun, Z; Marijanovic, Z; Marzo, L; Zurzolo, C, 2013) |
"Prion diseases are a group of lethal neurodegenerative disorders that develop as a result of conformational conversion of PrP(C) into PrP(Sc)." | ( Al-Ghamdi, S; Al-Masri, HT; Al-Talla, ZA; Emwas, AH; Guo, X, 2013) |
"Prion diseases are transmissible spongiform encephalopathies in humans and animals, including scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease (CWD) in deer, and Creutzfeldt-Jakob disease (CJD) in humans." | ( Bannach, O; Birkmann, E; Luers, L; Nagel-Steger, L; Riesner, D; Stöhr, J; Willbold, D; Wolff, M; Wördehoff, MM, 2013) |
"Prion diseases, or transmissible spongiform encephalopathies, are characterized by abnormal prion protein accumulation in the brain." | ( Doh-ura, K; Teruya, K, 2013) |
"Prion diseases are rare and aggressive neurodegenerative disorders caused by the accumulation of misfolded, toxic conformations of the prion protein (PrP)." | ( Casas-Tinto, S; Fernandez-Funez, P; Rincon-Limas, DE; Zhang, Y, 2014) |
"Prion diseases are a heterogeneous group of neurodegenerative disorders affecting various mammals including humans." | ( Chesebro, B; Moore, RA; Priola, SA; Sturdevant, DE, 2014) |
"Prion diseases are a family of progressive neurodegenerative disorders, which are fatal in the majority of cases and affect both humans and domestic animals." | ( Park, SY; Park, YG, 2014) |
"Prion diseases are fatal neurodegenerative diseases affecting mammals." | ( Banerjee, D; Sanyal, S, 2014) |
"Prion diseases are progressive neurodegenerative diseases that are associated with conformational changes that convert normal cellular prion protein (PrP(C)) into an abnormal pathogenic prion protein (PrP(Sc))." | ( Kawahara, M; Koyama, H; Mizuno, D; Ohkawara, S; Sadakane, Y, 2014) |
"Prion diseases are transmissible and fatal neurodegenerative disorders of humans and animals." | ( Cheng, YC; Corda, E; Gilch, S; Hannaoui, S; Shim, SY, 2014) |
"Prion diseases are neurodegenerative disorders characterized by the accumulation of an abnormal isoform of the protease-insensitive isoform (PrPSc) of prion protein." | ( Moon, JH; Park, SY, 2015) |
"Prion diseases are neurodegenerative disorders where infectious prion proteins (PrP) accumulate in brain leading to aggregation of amyloid fibrils and neuronal cell death." | ( Dash, D; Kumari, S; Mallick, RL; Singh, N; Sonkar, VK, 2015) |
"Prion diseases are a group of infectious and fatal neurodegenerative disorders caused by the conformational conversion of a cellular prion protein (PrP) into its abnormal isoform PrP(Sc)." | ( Du, W; He, L; Wang, X; Zhao, C; Zhu, D, 2015) |
"Prion diseases are caused by the conformational change of cellular prion protein PrP(C) into pathological prion protein PrP(Sc)." | ( Chen, J; Liang, Y; Pan, K; Yi, CW, 2015) |
"Prion diseases are fatal neurodegenerative disorders associated with the conversion of cellular prion protein (PrPC) into its aberrant infectious form (PrPSc)." | ( Campeau, J; Cortez, LM; Kalayil, M; McKenzie, D; Norman, G; Sim, VL; Van der Merwe, J, 2015) |
"Prion diseases are fatal neurodegenerative diseases that are transmissible to humans and other mammals." | ( Campeau, J; Cortez, LM; Kalayil, M; McKenzie, D; Norman, G; Sim, VL; Van der Merwe, J, 2015) |
"The pathogenesis of prion disease is the conversion of a normal type prion protein (PrP(C)) into a pathological isoform with protease resistance (PrP(Sc)), which accumulates in the brain." | ( Tsuboi, Y, 2015) |
"Prion diseases are fatal neurodegenerative diseases characterized by accumulation of the pathogenic prion protein PrP in the brain." | ( Giese, A; Griesinger, C; Leonov, A; Mitteregger-Kretzschmar, G; Ryazanov, S; Shi, S; Wagner, J, 2015) |
"Prion diseases are transmissible and invariably fatal neurodegenerative disorders, which still lack of efficacious prophylactic and therapeutic tools." | ( Chen, LN; Dong, XP; Shi, Q; Sun, J; Wang, H; Wang, J; Xiao, K; Zhang, BY; Zhang, J, 2016) |
"Prion diseases are characterized by the accumulation in the central nervous system of an abnormally folded isoform of the prion protein, named PrP(Sc)." | ( Alvarez-Martinez, MT; Ayrolles-Torro, A; Duarte Rodrigues, A; Imberdis, T; Kovacs, GG; Perrier, V; Robitzer, M; Torrent, J; Verdier, JM, 2016) |
"Prion diseases are fatal neurodegenerative disorders that are derived from structural changes of the native PrPc." | ( Lee, JH; Lee, YJ; Moon, JH; Park, SY, 2016) |
"Prion diseases are characterized biochemically by protein aggregation of infectious prion isoforms (PrPSc), which result from the conformational conversion of physiological prion proteins (PrPC)." | ( Brim, S; Groschup, MH; Kuczius, T, 2016) |
"Prion diseases are fatal, progressive, neurodegenerative diseases caused by prion accumulation in the brain and lymphoreticular system." | ( Doh-Ura, K; Kamitakahara, H; Kawata, M; Nishizawa, K; Oguma, A; Sakasegawa, Y; Teruya, K, 2016) |
"Human prion diseases are associated with a range of clinical presentations, and they are classified by both clinicopathological syndrome and etiology, with subclassification according to molecular criteria." | ( Adamson, G; Beck, JA; Brandner, S; Brock, L; Collinge, J; Joiner, S; Linehan, JM; Mead, S; Powell, C; Wadsworth, JDF, 2017) |
"Prion diseases are a group of incurable infectious terminal neurodegenerative diseases caused by the aggregated misfolded PrPsc in selected mammals including humans." | ( Bondy, SC; Prasad, KN, 2019) |
"Prion diseases are fatal transmissible neurodegenerative disorders that affect animals and humans." | ( Abdulrahman, BA; Doh-Ura, K; Gilch, S; Schatzl, HM; Tahir, W, 2019) |
"Human prion diseases are classified into sporadic, genetic, and acquired forms." | ( Brett, F; Calero, M; Calero, O; Catania, M; Collins, SJ; Ferrer, I; Heffernan, J; Hermann, P; Klotz, S; Kovacs, GG; Ladogana, A; Llorens, F; Moda, F; O'Regan, C; Pocchiari, M; Poleggi, A; Sarros, S; Schmitz, M; Stehmann, C; Villar-Piqué, A; Zerr, I, 2020) |
"Prion diseases are neurodegenerative diseases associated with neuron damage and behavioral disorders in animals and humans." | ( Ali Shah, SZ; Gao, H; Guan, Z; Lai, M; Li, J; Li, W; Wu, W; Yang, D; Yang, L; Zhang, X; Zhao, D; Zhao, H; Zhou, X, 2020) |
"Prion disease is a rapidly progressive neurodegenerative disorder caused by misfolding and aggregation of the prion protein (PrP), and there are currently no therapeutic options." | ( Auld, DS; Campbell, AJ; Casalena, D; Chan, AI; Dahlin, JL; Kaushik, VK; Leed, AJ; Lemke, CT; Liu, DR; Mesleh, MF; Minikel, EV; Moroco, JA; Nguyen, KT; Nonato, MC; Rangel, VL; Reidenbach, AG; Sacher, JR; Sathappa, M; Schreiber, SL; Shah, RN; Shrestha, OK; Usanov, DL; Vallabh, SM; Wagner, FF; Wright, SK; Yehl, JB, 2020) |
"Prion diseases are progressive and transmissive neurodegenerative diseases." | ( Kato-Negishi, M; Kawahara, M; Tanaka, KI, 2021) |
"Prion diseases are neurodegenerative disorders caused by conformational conversion of the cellular prion protein (PrPC) into scrapie prion protein (PrPSc)." | ( Aguzzi, A; Zhu, C, 2021) |
"Human prion diseases are fatal neurodegenerative disorders that include sporadic, infectious and genetic forms." | ( Beaudry, P; Bizat, N; Brandel, JP; Gougerot, A; Haïk, S; Laoues, S; Laplanche, JL; Levavasseur, E; Normant, S; Parrales, V; Privat, N; Ravassard, P; Roussel, J, 2021) |
"Genetic prion diseases are a rare and diverse group of fatal neurodegenerative disorders caused by pathogenic sequence variations in the prion protein gene, PRNP." | ( Baldeiras, I; Calero, M; Chen, C; Collins, S; Cramm, M; Dong, XP; Escaramís, G; Geschwind, MD; Golanska, E; Hermann, P; Kovacs, GG; Kruse, N; Ladogana, A; Lange, P; Liberski, PP; Llorens, F; Martí, E; Mitrová, E; Mollenhauer, B; Pocchiari, M; Poleggi, A; Sánchez-Valle, R; Santana, I; Sarros, S; Schmitz, M; Sikorska, B; Stehmann, C; Villar-Piqué, A; Žáková, D; Zerr, I, 2022) |
"Prion diseases are incurable, infectious and fatal neurodegenerative diseases that affect both humans and animals." | ( Ali, T; Cashion, J; Gilch, S; Zhang, W, 2023) |
"Prion Diseases or Transmissible Spongiform Encephalopathies are neurodegenerative conditions associated with a long incubation period and progressive clinical evolution, leading to death." | ( Batalha, PN; Boechat, FDCS; Costa, ARP; de Moraes, MC; de Souza, MCBV; Muxfeldt, M; Rangel, LP; Silva, JL; Vieira, TCRG, 2022) |
"Prion Diseases or Transmissible Spongiform Encephalopathies are neurodegenerative conditions associated with a long incubation period and progressive clinical evolution, leading to death." | ( Batalha, PN; Boechat, FDCS; Costa, ARP; de Moraes, MC; de Souza, MCBV; Muxfeldt, M; Rangel, LP; Silva, JL; Vieira, TCRG, 2022) |
"Prion Diseases or Transmissible Spongiform Encephalopathies are neurodegenerative conditions associated with a long incubation period and progressive clinical evolution, leading to death." | ( Batalha, PN; Boechat, FDCS; Costa, ARP; de Moraes, MC; de Souza, MCBV; Muxfeldt, M; Rangel, LP; Silva, JL; Vieira, TCRG, 2022) |
"Prion diseases are neurodegenerative disorders in humans and animals for which no therapies are currently available." | ( Gao, EM; Kim, CY; Kim, J; Kim, JH; Lee, H; Lee, S; Lee, Y; Ryou, C; Trinh, THT; Yoo, M, 2022) |
"Prion diseases are fatal and infectious neurodegenerative diseases that occur in humans and animals." | ( Cherry, P; Ebacher, V; Gilch, S; Hannaoui, S; Lu, L; Schatzl, HM; Shim, SY; Tahir, W, 2023) |
"Select prion diseases are characterized by widespread cerebral plaque-like deposits of amyloid fibrils enriched in heparan sulfate (HS), a abundant extracellular matrix component." | ( Aguilar-Calvo, P; Bapat, J; Barback, C; Caughey, B; Dwyer, CA; Esko, JD; Iliff, JJ; Kraus, A; Malik, A; Nilsson, KPR; Ogawa, R; Orrù, CD; Pizzo, DP; Riley, MB; Sandoval, DR; Sigurdson, CJ; Soldau, K; Standke, HG; Thomas, OR; Vera, DR, 2023) |