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Octarepeat region flexibility impacts prion function, endoproteolysis and disease manifestation.
Published in:
EMBO Molecular Medicine, 2015, v. 7, n. 3, p. 339, doi. 10.15252/emmm.201404588
By:
- Lau, Agnes;
- McDonald, Alex;
- Daude, Nathalie;
- Mays, Charles E;
- Walter, Eric D;
- Aglietti, Robin;
- Mercer, Robert CC;
- Wohlgemuth, Serene;
- Merwe, Jacques;
- Yang, Jing;
- Gapeshina, Hristina;
- Kim, Chae;
- Grams, Jennifer;
- Shi, Beipei;
- Wille, Holger;
- Balachandran, Aru;
- Schmitt‐Ulms, Gerold;
- Safar, Jiri G;
- Millhauser, Glenn L;
- Westaway, David
Publication type:
Article
Genome‐wide association studies identify novel loci in rapidly progressive Alzheimer's disease.
Published in:
Alzheimer's & Dementia: The Journal of the Alzheimer's Association, 2024, v. 20, n. 3, p. 2034, doi. 10.1002/alz.13655
By:
- Wang, Ping;
- Lynn, Audrey;
- Miskimen, Kristy;
- Song, Yeunjoo E.;
- Wisniewski, Thomas;
- Cohen, Mark;
- Appleby, Brian S.;
- Safar, Jiri G.;
- Haines, Jonathan L.
Publication type:
Article
Prion disease tempo determined by host-dependent substrate reduction.
Published in:
Journal of Clinical Investigation, 2014, v. 124, n. 2, p. 847, doi. 10.1172/JCI72241
By:
- Mays, Charles E.;
- Chae Kim;
- Haldiman, Tracy;
- van der Merwe, Jacques;
- Lau, Agnes;
- Jing Yang;
- Grams, Jennifer;
- Di Bari, Michele A.;
- Nonno, Romolo;
- Telling, Glenn C.;
- Qingzhong Kong;
- Langeveld, Jan;
- McKenzie, Debbie;
- Westaway, David;
- Safar, Jiri G.
Publication type:
Article
Evolving prion-like tau conformers differentially alter postsynaptic proteins in neurons inoculated with distinct isolates of Alzheimer's disease tau.
Published in:
Cell & Bioscience, 2023, v. 13, n. 1, p. 1, doi. 10.1186/s13578-023-01133-0
By:
- Hromadkova, Lenka;
- Kim, Chae;
- Haldiman, Tracy;
- Peng, Lihua;
- Zhu, Xiongwei;
- Cohen, Mark;
- de Silva, Rohan;
- Safar, Jiri G.
Publication type:
Article
Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice.
Published in:
Nature Biotechnology, 2002, v. 20, n. 11, p. 1147, doi. 10.1038/nbt748
By:
- Safar, Jiri G.;
- Scott, Michael;
- Monaghan, Jeff;
- Deering, Camille;
- Didorenko, Svetlana;
- Vergara, Julie;
- Ball, Haydn;
- Legname, Giuseppe;
- Leclerc, Estelle;
- Solforosi, Laura;
- Serban, Hana;
- Groth, Darlene;
- Burton, Dennis R.;
- Prusiner, Stanley B.;
- Williamson, R. Anthony
Publication type:
Article
Pathologic tau conformer ensembles induce dynamic, liquid-liquid phase separation events at the nuclear envelope.
Published in:
BMC Biology, 2021, v. 19, n. 1, p. 1, doi. 10.1186/s12915-021-01132-y
By:
- Kang, Sang-Gyun;
- Han, Zhuang Zhuang;
- Daude, Nathalie;
- McNamara, Emily;
- Wohlgemuth, Serene;
- Molina-Porcel, Laura;
- Safar, Jiri G.;
- Mok, Sue-Ann;
- Westaway, David
Publication type:
Article
Novel strain properties distinguishing sporadic prion diseases sharing prion protein genotype and prion type.
Published in:
Scientific Reports, 2017, p. 38280, doi. 10.1038/srep38280
By:
- Cracco, Laura;
- Notari, Silvio;
- Cali, Ignazio;
- Sy, Man-Sun;
- Chen, Shu G.;
- Cohen, Mark L.;
- Ghetti, Bernardino;
- Appleby, Brian S.;
- Zou, Wen-Quan;
- Caughey, Byron;
- Safar, Jiri G.;
- Gambetti, Pierluigi
Publication type:
Article
Transmission and Detection of Prions in Feces.
Published in:
Journal of Infectious Diseases, 2008, v. 198, n. 1, p. 80, doi. 10.1086/588193
By:
- Safar, Jiri G.;
- Lessard, Pierre;
- Tamgüney, Gültekin;
- Freyman, Yevgeniy;
- Deering, Camille;
- Letessier, Frederic;
- DeArmond, Stephen J.;
- Prusiner, Stanley B.
Publication type:
Article
Structurally distinct external solvent-exposed domains drive replication of major human prions.
Published in:
PLoS Pathogens, 2021, v. 17, n. 6, p. 1, doi. 10.1371/journal.ppat.1009642
By:
- Siddiqi, Mohammad Khursheed;
- Kim, Chae;
- Haldiman, Tracy;
- Kacirova, Miroslava;
- Wang, Benlian;
- Bohon, Jen;
- Chance, Mark R.;
- Kiselar, Janna;
- Safar, Jiri G.
Publication type:
Article
Evaluation of a New Criterion for Detecting Prion Disease With Diffusion Magnetic Resonance Imaging.
Published in:
JAMA Neurology, 2020, v. 77, n. 9, p. 1141, doi. 10.1001/jamaneurol.2020.1319
By:
- Bizzi, Alberto;
- Pascuzzo, Riccardo;
- Blevins, Janis;
- Grisoli, Marina;
- Lodi, Raffaele;
- Moscatelli, Marco E. M.;
- Castelli, Gianmarco;
- Cohen, Mark L.;
- Schonberger, Lawrence B.;
- Foutz, Aaron;
- Safar, Jiri G.;
- Appleby, Brian S.;
- Gambetti, Pierluigi
Publication type:
Article
Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics.
Published in:
2009
By:
- Cali I;
- Castellani R;
- Alshekhlee A;
- Cohen Y;
- Blevins J;
- Yuan J;
- Langeveld JP;
- Parchi P;
- Safar JG;
- Zou WQ;
- Gambetti P;
- Cali, Ignazio;
- Castellani, Rudolph;
- Alshekhlee, Amer;
- Cohen, Yvonne;
- Blevins, Janis;
- Yuan, Jue;
- Langeveld, Jan P M;
- Parchi, Piero;
- Safar, Jiri G
Publication type:
journal article
Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt–Jakob disease: its effect on the phenotype and prion-type characteristics.
Published in:
Brain: A Journal of Neurology, 2009, v. 132, n. 10, p. 2643, doi. 10.1093/brain/awp196
By:
- Cali, Ignazio;
- Castellani, Rudolph;
- Alshekhlee, Amer;
- Cohen, Yvonne;
- Blevins, Janis;
- Yuan, Jue;
- Langeveld, Jan P. M.;
- Parchi, Piero;
- Safar, Jiri G.;
- Zou, Wen-Quan;
- Gambetti, Pierluigi
Publication type:
Article
Ring trial of 2nd generation RT‐QuIC diagnostic tests for sporadic CJD.
Published in:
Annals of Clinical & Translational Neurology, 2020, v. 7, n. 11, p. 2262, doi. 10.1002/acn3.51219
By:
- Orrú, Christina D.;
- Groveman, Bradley R.;
- Foutz, Aaron;
- Bongianni, Matilde;
- Cardone, Franco;
- McKenzie, Neil;
- Culeux, Audrey;
- Poleggi, Anna;
- Grznarova, Katarina;
- Perra, Daniela;
- Fiorini, Michele;
- Liu, Xiaoqin;
- Ladogana, Anna;
- Sbriccoli, Marco;
- Hughson, Andrew G.;
- Haïk, Stéphane;
- Green, Alison J.;
- Geschwind, Michael D.;
- Pocchiari, Maurizio;
- Safar, Jiri G.
Publication type:
Article
Prion seeding activity and infectivity in skin samples from patients with sporadic Creutzfeldt-Jakob disease.
Published in:
Science Translational Medicine, 2017, v. 9, n. 417, p. 1, doi. 10.1126/scitranslmed.aam7785
By:
- Orrú, Christina D.;
- Jue Yuan;
- Appleby, Brian S.;
- Baiya Li;
- Yu Li;
- Winner, Dane;
- Zerui Wang;
- Yi-An Zhan;
- Rodgers, Mark;
- Rarick, Jason;
- Wyza, Robert E.;
- Joshi, Tripti;
- Gong-Xian Wang;
- Cohen, Mark L.;
- Shulin Zhang;
- Groveman, Bradley R.;
- Petersen, Robert B.;
- Ironside, James W.;
- Quiñones-Mateu, Miguel E.;
- Safar, Jiri G.
Publication type:
Article
Diagnostic and prognostic value of human prion detection in cerebrospinal fluid.
Published in:
2017
By:
- Foutz, Aaron;
- Appleby, Brian S.;
- Hamlin, Clive;
- Liu, Xiaoqin;
- Yang, Sheng;
- Cohen, Yvonne;
- Chen, Wei;
- Blevins, Janis;
- Fausett, Cameron;
- Wang, Han;
- Gambetti, Pierluigi;
- Zhang, Shulin;
- Hughson, Andrew;
- Tatsuoka, Curtis;
- Schonberger, Lawrence B.;
- Cohen, Mark L.;
- Caughey, Byron;
- Safar, Jiri G.
Publication type:
journal article
Structural Determinants of Phenotypic Diversity and Replication Rate of Human Prions.
Published in:
PLoS Pathogens, 2015, v. 11, n. 4, p. 1, doi. 10.1371/journal.ppat.1004832
By:
- Kabir, Mohammad E.;
- Kim, Chae;
- Haldiman, Tracy;
- Safar, Jiri G.;
- Cohen, Mark L.;
- Cohen, Yvonne;
- Chen, Wei;
- Xiao, Xiangzhu;
- Chen, Shugui;
- Surewicz, Witold K.
Publication type:
Article
Small Protease Sensitive Oligomers of PrPSc in Distinct Human Prions Determine Conversion Rate of PrPC.
Published in:
PLoS Pathogens, 2012, v. 8, n. 8, p. 1, doi. 10.1371/journal.ppat.1002835
By:
- Chae Kim;
- Haldiman, Tracy;
- Surewicz, Krystyna;
- Cohen, Yvonne;
- Wei Chen;
- Blevins, Janis;
- Man-Sun Sy;
- Cohen, Mark;
- Qingzhong Kong;
- Telling, Glenn C.;
- Surewicz, Witold K.;
- Safar, Jiri G.
Publication type:
Article
Protease-Sensitive Conformers in Broad Spectrum of Distinct PrPSc Structures in Sporadic Creutzfeldt-Jakob Disease Are Indicator of Progression Rate.
Published in:
PLoS Pathogens, 2011, v. 7, n. 9, p. 1, doi. 10.1371/journal.ppat.1002242
By:
- Chae Kim;
- Haldiman, Tracy;
- Cohen, Yvonne;
- Wei Chen;
- Blevins, Janis;
- Man-Sun Sy;
- Cohen, Mark;
- Safar, Jiri G.
Publication type:
Article
Cellular Biology of Tau Diversity and Pathogenic Conformers.
Published in:
Frontiers in Neurology, 2020, v. 11, p. N.PAG, doi. 10.3389/fneur.2020.590199
By:
- Kang, Sang-Gyun;
- Eskandari-Sedighi, Ghazaleh;
- Hromadkova, Lenka;
- Safar, Jiri G.;
- Westaway, David
Publication type:
Article
A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles.
Published in:
PLoS Pathogens, 2018, v. 14, n. 1, p. 1, doi. 10.1371/journal.ppat.1006826
By:
- Mercer, Robert C. C.;
- Daude, Nathalie;
- Dorosh, Lyudmyla;
- Fu, Ze-Lin;
- Mays, Charles E.;
- Gapeshina, Hristina;
- Wohlgemuth, Serene L.;
- Acevedo-Morantes, Claudia Y.;
- Yang, Jing;
- Cashman, Neil R.;
- Coulthart, Michael B.;
- Pearson, Dawn M.;
- Joseph, Jeffrey T.;
- Wille, Holger;
- Safar, Jiri G.;
- Jansen, Gerard H.;
- Stepanova, Maria;
- Sykes, Brian D.;
- Westaway, David
Publication type:
Article
Neuropathologically directed profiling of PRNP somatic and germline variants in sporadic human prion disease.
Published in:
Acta Neuropathologica, 2024, v. 148, n. 1, p. 1, doi. 10.1007/s00401-024-02774-2
By:
- McDonough, Gannon A.;
- Cheng, Yuchen;
- Morillo, Katherine S.;
- Doan, Ryan N.;
- Zhou, Zinan;
- Kenny, Connor J.;
- Foutz, Aaron;
- Kim, Chae;
- Cohen, Mark L.;
- Appleby, Brian S.;
- Walsh, Christopher A.;
- Safar, Jiri G.;
- Huang, August Yue;
- Miller, Michael B.
Publication type:
Article
Correction to: Diverse, evolving conformer populations drive distinct phenotypes in frontotemporal lobar degeneration caused by the same MAPT‑P301L mutation.
Published in:
2021
By:
- Daude, Nathalie;
- Kim, Chae;
- Kang, Sang‑Gyun;
- Eskandari‑Sedighi, Ghazaleh;
- Haldiman, Tracy;
- Yang, Jing;
- Fleck, Shelaine C.;
- Gomez‑Cardona, Erik;
- Han, Zhuang Zhuang;
- Borrego‑Ecija, Sergi;
- Wohlgemuth, Serene;
- Julien, Olivier;
- Wille, Holger;
- Molina‑Porcel, Laura;
- Gelpi, Ellen;
- Safar, Jiri G.;
- Westaway, David
Publication type:
Correction Notice
Diverse, evolving conformer populations drive distinct phenotypes in frontotemporal lobar degeneration caused by the same MAPT-P301L mutation.
Published in:
Acta Neuropathologica, 2020, v. 139, n. 6, p. 1045, doi. 10.1007/s00401-020-02148-4
By:
- Daude, Nathalie;
- Kim, Chae;
- Kang, Sang-Gyun;
- Eskandari-Sedighi, Ghazaleh;
- Haldiman, Tracy;
- Yang, Jing;
- Fleck, Shelaine C.;
- Gomez-Cardona, Erik;
- Han, Zhuang Zhuang;
- Borrego-Ecija, Sergi;
- Wohlgemuth, Serene;
- Julien, Olivier;
- Wille, Holger;
- Molina-Porcel, Laura;
- Gelpi, Ellen;
- Safar, Jiri G.;
- Westaway, David
Publication type:
Article
Shortening heparan sulfate chains prolongs survival and reduces parenchymal plaques in prion disease caused by mobile, ADAM10-cleaved prions.
Published in:
Acta Neuropathologica, 2020, v. 139, n. 3, p. 527, doi. 10.1007/s00401-019-02085-x
By:
- Aguilar-Calvo, Patricia;
- Sevillano, Alejandro M.;
- Bapat, Jaidev;
- Soldau, Katrin;
- Sandoval, Daniel R.;
- Altmeppen, Hermann C.;
- Linsenmeier, Luise;
- Pizzo, Donald P.;
- Geschwind, Michael D.;
- Sanchez, Henry;
- Appleby, Brian S.;
- Cohen, Mark L.;
- Safar, Jiri G.;
- Edland, Steven D.;
- Glatzel, Markus;
- Nilsson, K. Peter R.;
- Esko, Jeffrey D.;
- Sigurdson, Christina J.
Publication type:
Article
Populations of Tau Conformers Drive Prion-like Strain Effects in Alzheimer's Disease and Related Dementias.
Published in:
Cells (2073-4409), 2022, v. 11, n. 19, p. 2997, doi. 10.3390/cells11192997
By:
- Hromadkova, Lenka;
- Siddiqi, Mohammad Khursheed;
- Liu, He;
- Safar, Jiri G.
Publication type:
Article
Artificial strain of human prions created in vitro.
Published in:
Nature Communications, 2018, v. 9, n. 1, p. 1, doi. 10.1038/s41467-018-04584-z
By:
- Chae Kim;
- Xiangzhu Xiao;
- Shugui Chen;
- Haldiman, Tracy;
- Smirnovas, Vitautas;
- Kofskey, Diane;
- Warren, Miriam;
- Surewicz, Krystyna;
- Maurer, Nicholas R.;
- Qingzhong Kong;
- Surewicz, Witold;
- Safar, Jiri G.
Publication type:
Article
A Role of Low-Density Lipoprotein Receptor-Related Protein 4 (LRP4) in Astrocytic Aβ Clearance.
Published in:
Journal of Neuroscience, 2020, v. 40, n. 28, p. 5347, doi. 10.1523/JNEUROSCI.0250-20.2020
By:
- Hongsheng Zhang;
- Wenbing Chen;
- Zhibing Tan;
- Lei Zhang;
- Zhaoqi Dong;
- Wanpeng Cui;
- Kai Zhao;
- Hongsheng Wang;
- Hongyang Jing;
- Rangjuan Cao;
- Chae Kim;
- Safar, Jiri G.;
- Wen-Cheng Xiong;
- Lin Mei
Publication type:
Article
Distinct populations of highly potent TAU seed conformers in rapidly progressing Alzheimer's disease.
Published in:
Science Translational Medicine, 2022, v. 14, n. 626, p. 1, doi. 10.1126/scitranslmed.abg0253
By:
- Kim, Chae;
- Haldiman, Tracy;
- Kang, Sang-Gyun;
- Hromadkova, Lenka;
- Han, Zhuang Zhuang;
- Chen, Wei;
- Lissemore, Frances;
- Lerner, Alan;
- de Silva, Rohan;
- Cohen, Mark L.;
- Westaway, David;
- Safar, Jiri G.
Publication type:
Article
Detection of prions in matching post-mortem skin and cerebrospinal fluid samples using second-generation real-time quaking-induced conversion assay.
Published in:
Scientific Reports, 2024, v. 14, n. 1, p. 1, doi. 10.1038/s41598-024-56789-6
By:
- Baranová, Soňa;
- Moško, Tibor;
- Brůžová, Magdalena;
- Haldiman, Tracy;
- Kim, Chae;
- Safar, Jiri G.;
- Matěj, Radoslav;
- Holada, Karel
Publication type:
Article

Found: 29

Octarepeat region flexibility impacts prion function, endoproteolysis and disease manifestation.

Genome‐wide association studies identify novel loci in rapidly progressive Alzheimer's disease.

Prion disease tempo determined by host-dependent substrate reduction.

Evolving prion-like tau conformers differentially alter postsynaptic proteins in neurons inoculated with distinct isolates of Alzheimer's disease tau.

Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice.

Pathologic tau conformer ensembles induce dynamic, liquid-liquid phase separation events at the nuclear envelope.

Novel strain properties distinguishing sporadic prion diseases sharing prion protein genotype and prion type.

Transmission and Detection of Prions in Feces.

Structurally distinct external solvent-exposed domains drive replication of major human prions.

Evaluation of a New Criterion for Detecting Prion Disease With Diffusion Magnetic Resonance Imaging.

Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics.

Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt–Jakob disease: its effect on the phenotype and prion-type characteristics.

Ring trial of 2nd generation RT‐QuIC diagnostic tests for sporadic CJD.

Prion seeding activity and infectivity in skin samples from patients with sporadic Creutzfeldt-Jakob disease.

Diagnostic and prognostic value of human prion detection in cerebrospinal fluid.

Structural Determinants of Phenotypic Diversity and Replication Rate of Human Prions.

Small Protease Sensitive Oligomers of PrP<sup>Sc</sup> in Distinct Human Prions Determine Conversion Rate of PrP<sup>C</sup>.

Protease-Sensitive Conformers in Broad Spectrum of Distinct PrP<sup>Sc</sup> Structures in Sporadic Creutzfeldt-Jakob Disease Are Indicator of Progression Rate.

Cellular Biology of Tau Diversity and Pathogenic Conformers.

A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles.

Neuropathologically directed profiling of PRNP somatic and germline variants in sporadic human prion disease.

Correction to: Diverse, evolving conformer populations drive distinct phenotypes in frontotemporal lobar degeneration caused by the same MAPT‑P301L mutation.

Diverse, evolving conformer populations drive distinct phenotypes in frontotemporal lobar degeneration caused by the same MAPT-P301L mutation.

Shortening heparan sulfate chains prolongs survival and reduces parenchymal plaques in prion disease caused by mobile, ADAM10-cleaved prions.

Populations of Tau Conformers Drive Prion-like Strain Effects in Alzheimer's Disease and Related Dementias.

Artificial strain of human prions created in vitro.

A Role of Low-Density Lipoprotein Receptor-Related Protein 4 (LRP4) in Astrocytic Aβ Clearance.

Distinct populations of highly potent TAU seed conformers in rapidly progressing Alzheimer's disease.

Detection of prions in matching post-mortem skin and cerebrospinal fluid samples using second-generation real-time quaking-induced conversion assay.