Compounds > methionine
Page last updated: 2024-08-17

methionine and Creutzfeldt-Jakob Syndrome

methionine has been researched along with Creutzfeldt-Jakob Syndrome in 74 studies

Research

Studies (74)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's6 (8.11)18.2507
2000's36 (48.65)29.6817
2010's23 (31.08)24.3611
2020's9 (12.16)2.80

Authors

AuthorsStudies
Appleby, BS; Brennecke, N; Cali, I; Cohen, ML; Collins, SJ; Cracco, L; Hosszu, LLP; Mead, S; Mok, TH; Prior, TW; Puoti, G; Speedy, H; Stehmann, C1
Ae, R; Hamaguchi, T; Kitamoto, T; Kosami, K; Mizusawa, H; Nakamura, Y; Sanjo, N; Tsukamoto, T; Yamada, M1
Bolakhrif, N; Gohlke, H; Gremer, L; Kaiser, J; Nagel-Steger, L; Pauly, T; Willbold, D1
Aiba, I; Akagi, A; Hashimoto, R; Inukai, A; Iwasaki, Y; Kitamoto, T; Mimuro, M; Miyahara, H; Riku, Y; Yoshida, M1
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, I1
Appleby, BS; Bharara Singh, A; Cali, I; Cracco, L; Gambetti, P; Lavrich, J; Nemani, SK; Nigro, M; Notari, S; Puoti, G; Sim, VL; Surewicz, WK; Xiao, X1
Belondrade, M; Bouaziz-Amar, E; Bougard, D; Brandel, JP; Culeux, A; Denouel, A; Grznarova, K; Haïk, S; Levasseur, M; Plu, I; Seilhean, D; Vlaicu, MB1
Akagi, A; Ito, M; Iwasaki, Y; Kawai, Y; Kitamoto, T; Kobayashi, A; Miyahara, H; Mori, K; Riku, Y; Yoshida, M1
Kaneda, D; Kitamoto, T; Kusaka, H; Nakamura, T; Shinde, A; Shintaku, M; Takeuchi, A1
Akagi, A; Iwasaki, Y; Kitamoto, T; Mimuro, M; Yamada, M; Yoshida, M1
Barria, MA; Green, AJ; Head, MW; Knight, R; Lee, A1
Bélondrade, M; Bougard, D; Bruyère-Ostells, L; Fournier-Wirth, C; Green, AJE; Knight, RS; Lehmann, S; Mayran, C; Will, RG1
Ito, M; Iwasaki, Y; Kawai, Y; Mori, K1
Beekes, M; Ebert, E; Lüllmann, K; Mitrova, E; Oikonomou, P; Schlomm, M; Schmitz, M; Wohlhage, M; Zafar, S; Zerr, I1
Kim, YS; Lee, A; Lim, JG; Oh, E; Park, S1
Brown, P; Kitamoto, T; Kobayashi, A; Matsuura, Y; Mohri, S; Parchi, P; Saverioni, D; Takeuchi, A; Yamada, M1
Berghoff, AS; Hortobágyi, T; Kovacs, GG; Ströbel, T; Trummert, A; Unterberger, U1
Iwaki, T; Iwasaki, Y; Kato, S; Kitamoto, T; Kobayashi, A; Matsuura, Y; Mohri, S; Murayama, S; Takahashi, H; Takao, M; Yamada, M; Yoshida, M1
Adlard, P; Bjurstrom, N; Brandner, S; Caine, D; Collinge, J; Druyeh, R; Hummerich, H; Hyare, H; Jaunmuktane, Z; Lowe, J; Mead, S; Norsworthy, P; Rudge, P; Wadsworth, JD1
Kitamoto, T; Kobayashi, A; Mohri, S; Parchi, P; Yamada, M1
Feng, B; Wang, Z; Xiao, G; Zhou, Z1
Choi, YP; Head, MW; Ironside, JW; Moore, RA; Priola, SA; Pyo Choi, Y; Ritchie, DL; Zanusso, G1
Kitamoto, T; Kobayashi, A; Morita, M; Parchi, P; Takeuchi, A; Uno, S; Yamada, M1
Agrimi, U; Cardone, F; Di Bari, MA; Fiorini, M; Galeno, R; Graziano, S; Ingrosso, L; Ladogana, A; Monaco, S; Nonno, R; Pasini, G; Pocchiari, M; Poleggi, A; Puopolo, M; Sbriccoli, M; Valanzano, A; Vinci, R; Zanusso, G1
Diéterlen, F; Lucotte, G; Mercier, G1
Corcia, P; De Toffol, B; Guennoc, AM; Haïk, S; Limousin, N; Lucas, B; Praline, J; Sazdovitch, V1
Pocchiari, M1
Capellari, S; Cras, P; Ghetti, B; Giese, A; Kretzschmar, H; Ladogana, A; Langeveld, JP; Notari, S; Parchi, P; Pocchiari, M; Roncaroli, F; Strammiello, R; Zerr, I1
Alshekhlee, A; Blevins, J; Cali, I; Castellani, R; Cohen, Y; Gambetti, P; Langeveld, JP; Parchi, P; Safar, JG; Yuan, J; Zou, WQ1
Béjot, Y; Caillier, M; Giroud, M; Laplanche, JL; Moreau, T; Osseby, GV1
Andrews, MM; Bishop, MT; Chohan, G; Green, AJ; Knight, RS; McCord, Y; McKenzie, JM; Pennington, C1
Bernovská, V; Cernák, A; Cernák, M; Koščová, S; Mitrová, E; Slivarichová, D1
Ito, D; Kitamoto, T; Nishimoto, Y; Shimizu, T; Suzuki, N; Suzuki, S1
Chen, RP; Lee, LY; Liao, TY1
Blevins, J; Chen, W; Cohen, M; Cohen, Y; Haldiman, T; Kim, C; Safar, JG; Sy, MS1
Ironside, JW; Knight, R; Liberski, PP; Sikorska, B1
Dong, XP; Gong, HS; Guo, Y; Liu, Y; Shi, Q; Tian, C; Wang, SB; Xia, SL; Xie, WL; Xu, BL; Xu, Y; Zhang, BY; Zhang, J1
Belay, G; Mitrová, E1
Beaudry, P; Capellari, S; Dartigues, JF; Delasnerie-Lauprêtre, N; Desbordes, P; Gambetti, P; Laplanche, JL; Mary, JY; Parchi, P; Peoc'h, K; Vital, A; Vital, C1
Belay, G; Budka, H; Hainfellner, JA; Jarius, C; Kovacs, GG; Mitrova, E1
Alpers, M; Beck, JA; Campbell, T; Collinge, J; Fisher, EM; Goldstein, D; Mead, S; Poulter, M; Stumpf, MP; Uphill, JB; Whitfield, J1
Bonn, D1
Batchelor, M; Bhelt, D; Clarke, AR; Collinge, J; Hosszu, LL; Jackson, GS; Jones, S; Prodromidou, K; Trevitt, CR; Waltho, JP1
Disterer, P; Gill, AC; James, W; Tahiri-Alaoui, A1
Ward, HJ; Will, RG1
Cervenakova, L; Gajdusek, DC; Goldfarb, LG1
Chrétien, F; Créminon, C; Delisle, MB; Dormont, D; Gambetti, P; Gras, G; Gray, F; Ironside, JW; Le Pavec, G; Mikol, J; Parchi, P; Uro-Coste, E; Vallat-Decouvelaere, AV1
Asante, EA; Brandner, S; Collinge, J; Desbruslais, M; Gowland, I; Hill, AF; Joiner, S; Linehan, JM; Lloyd, SE; Stone, L; Wadsworth, JD; Welch, J1
Carrell, RW1
Ironside, JW; Kitamoto, T; Kobayashi, A; Mohri, S; Satoh, S1
Bradbury, J1
Baruzzi, A; Capellari, S; Cardone, F; Maras, B; Notari, S; Parchi, P; Pocchiari, M; Schininà, ME; Sità, D1
Bell, KL; Chen, SG; Chin, SS; Dong, Z; Gambetti, P; Pastore, M; Yang, L; Yang, Q; Yuan, J; Zou, WQ1
Karlou, M; Korkolopoulou, P; Kouzoupis, A; Malamis, G; Mazmanian, N; Michalopoulos, NV; Papanastasiou, PI; Patsouris, E; Saetta, AA1
Li, H; Lo, RY; Shyu, WC1
Boelle, PY; Cesbron, JY; Chatignoux, E; Valleron, AJ1
Inada, H; Kato, R; Kobayashi, M; Nagasaka, T; Nitta, K; Ohta, E; Shimokawa, C; Shindo, K; Shiozawa, Z; Togashi, S1
Kamitani, T; Kawauchi, Y; Kishida, H; Kitamoto, T; Yagishita, S1
Bratosiewicz-Wasik, J; Golanska, E; Jansen, GH; Liberski, PP; Wasik, TJ1
Antunes, H; Baldeiras, I; Carpenter, S; Castro, L; Ferreira, C; Machado, A; Magalhães, Z; Ramalheira, J; Ribeiro, MH; Santana, I; Soares, H1
Doh-ura, K; Fujihara, K; Hidaka, M; Itoyama, Y; Kanno, S; Kim, K; Kitamoto, T; Konishi, T; Konno, H; Kuroda, S; Matsuda, Y; Mizusawa, H; Nagasato, K; Nakamura, Y; Nakashima, I; Nobukuni, K; Sano, Y; Sasaki, K; Sato, S; Sato, T; Satoh, A; Satoh, K; Shiga, Y; Takahashi, H; Takano, H; Takata, H; Umeda, Y; Yamada, M1
Binelli, S; Bugiani, O; Capobianco, R; Di Fede, G; Fociani, P; Giaccone, G; Grisoli, M; Limido, L; Mangieri, M; Suardi, S; Tagliavini, F1
Sasaki, S1
Brandel, JP; Chiras, J; Dormont, D; Galanaud, D; Haïk, S; Ranjeva, JP1
Bell, J; de Silva, R; Dempster, M; Esmonde, T; Estibeiro, P; Ironside, JW; Lathe, R; McCardle, L; Will, R; Windl, O1
Brown, P; Cervenáková, L; Goldfarb, LG; Martinez-Lage, JF; Masullo, C; McCombie, WR; Pocchiari, M; Rubenstein, R; Scalici, C; Will, RG1
Capellari, S; Castellani, R; Chen, SG; Dickson, DW; Farlow, M; Gambetti, P; Ghetti, B; Parchi, P; Petersen, RB; Sima, AA; Trojanowski, JQ; Young, K1
Brown, P; Cervenakova, L; Chaunu, MP; el Hachimi, KH; Foncin, JF1
Brown, P; Cervenáková, L; Gajdusek, DC; Garruto, R; Goldfarb, LG; Lee, HS1
Alperovitch, A; Collins, S; de Pedro Cuesta, J; Hegyi, I; Kretzschmar, H; Mitrova, E; Pocchiari, M; van Duijn, C; Will, RG; Zerr, I1
Cousens, SN; Estibeiro, K; Green, AJ; Ironside, JW; Knight, RS; Mackenzie, J; Macleod, MA; Stewart, GE; Will, RG; Zeidler, M1
Alpérovitch, A; Brandel, JP; d'Aignaux, JH; Delasnerie-Lauprêtre, N; Hauw, JJ; Laplanche, JL; Peoc'h, K; Salomon, D1
Cousens, SN; d'Aignaux, JN; Smith, PG1
Head, MW; Horsburgh, A; Ironside, JW; Lim, Z; McCardle, L1

Reviews

6 review(s) available for methionine and Creutzfeldt-Jakob Syndrome

ArticleYear
Neuropathological and biochemical criteria to identify acquired Creutzfeldt-Jakob disease among presumed sporadic cases.
    Neuropathology : official journal of the Japanese Society of Neuropathology, 2016, Volume: 36, Issue:3

    Topics: Animals; Brain; Codon; Creutzfeldt-Jakob Syndrome; Disease Models, Animal; Gene Knock-In Techniques; Genotype; Humans; Methionine; Mice; Mice, Transgenic; Phenotype; Prion Proteins; PrPC Proteins; PrPSc Proteins

2016
Creutzfeldt-Jakob disease.
    Advances in experimental medicine and biology, 2012, Volume: 724

    Topics: Animals; Cattle; Creutzfeldt-Jakob Syndrome; Humans; Methionine; Molecular Weight; Plaque, Amyloid; Prion Proteins; Prions

2012
Clinical features of variant Creutzfeldt-Jakob disease.
    Current topics in microbiology and immunology, 2004, Volume: 284

    Topics: Age Factors; Cognition Disorders; Creutzfeldt-Jakob Syndrome; Diagnosis, Differential; Humans; Methionine; Prions

2004
Genetic studies in relation to kuru: an overview.
    Current molecular medicine, 2004, Volume: 4, Issue:4

    Topics: Animals; Cattle; Creutzfeldt-Jakob Syndrome; Genetic Predisposition to Disease; Genotype; Humans; Kuru; Methionine; Papua New Guinea; Phenotype; Prions; Risk Factors

2004
[Attention-getting zoonoses: Variant CJD].
    Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine, 2007, Nov-10, Volume: 96, Issue:11

    Topics: Animals; Brain; Cattle; Codon; Creutzfeldt-Jakob Syndrome; Homozygote; Humans; Magnetic Resonance Imaging; Methionine; PrPC Proteins; PrPSc Proteins; Zoonoses

2007
Pathological diagnosis of variant Creutzfeldt-Jakob disease.
    APMIS : acta pathologica, microbiologica, et immunologica Scandinavica, 2002, Volume: 110, Issue:1

    Topics: Autopsy; Biopsy; Blotting, Western; Brain; Creutzfeldt-Jakob Syndrome; Frontal Lobe; Homozygote; Humans; Immunohistochemistry; Lymphoid Tissue; Methionine; PrPSc Proteins

2002

Other Studies

68 other study(ies) available for methionine and Creutzfeldt-Jakob Syndrome

ArticleYear
Characterization of Prion Disease Associated with a Two-Octapeptide Repeat Insertion.
    Viruses, 2021, 09-08, Volume: 13, Issue:9

    Topics: Aged; Aged, 80 and over; Alleles; Brain; Creutzfeldt-Jakob Syndrome; Female; Humans; Male; Methionine; Middle Aged; Mutagenesis, Insertional; Oligopeptides; Prion Diseases; Prion Proteins; Prions

2021
Methionine homozygosity for PRNP polymorphism and susceptibility to human prion diseases.
    Journal of neurology, neurosurgery, and psychiatry, 2022, Volume: 93, Issue:7

    Topics: Animals; Case-Control Studies; Cattle; Codon; Creutzfeldt-Jakob Syndrome; Encephalopathy, Bovine Spongiform; Humans; Methionine; Prion Diseases; Prion Proteins; Prions

2022
Met/Val129 polymorphism of the full-length human prion protein dictates distinct pathways of amyloid formation.
    The Journal of biological chemistry, 2022, Volume: 298, Issue:10

    Topics: Amyloid; Amyloidosis; Creutzfeldt-Jakob Syndrome; Humans; Insomnia, Fatal Familial; Methionine; Polymorphism, Genetic; Prion Proteins; Protein Folding; Valine

2022
A case of M232R genetic Creutzfeldt-Jakob disease with Lewy bodies.
    Journal of the neurological sciences, 2020, 02-15, Volume: 409

    Topics: Aged; Arginine; Creutzfeldt-Jakob Syndrome; Diagnosis, Differential; Fatal Outcome; Humans; Lewy Body Disease; Male; Methionine; Prion Proteins

2020
Diagnostic Accuracy of Prion Disease Biomarkers in Iatrogenic Creutzfeldt-Jakob Disease.
    Biomolecules, 2020, 02-12, Volume: 10, Issue:2

    Topics: Adult; Aged; Biomarkers; Corneal Transplantation; Creutzfeldt-Jakob Syndrome; Dura Mater; Electroencephalography; Encephalopathy, Bovine Spongiform; Female; Homozygote; Human Growth Hormone; Humans; Iatrogenic Disease; Kaplan-Meier Estimate; Magnetic Resonance Imaging; Male; Methionine; Middle Aged; Neuroimaging; Phenotype; Polymorphism, Genetic; Prion Diseases; Prion Proteins; Registries; Reproducibility of Results; Sex Factors; Time Factors

2020
A novel mechanism of phenotypic heterogeneity in Creutzfeldt-Jakob disease.
    Acta neuropathologica communications, 2020, 06-19, Volume: 8, Issue:1

    Topics: Cerebellum; Cerebral Cortex; Creutzfeldt-Jakob Syndrome; Epitope Mapping; Humans; Mass Spectrometry; Methionine; Phenotype; Prion Proteins; Valine

2020
Variant Creutzfeldt-Jakob Disease Diagnosed 7.5 Years after Occupational Exposure.
    The New England journal of medicine, 2020, 07-02, Volume: 383, Issue:1

    Topics: Accidents, Occupational; Adult; Animals; Brain; Brain Chemistry; Creutzfeldt-Jakob Syndrome; Fatal Outcome; Female; Humans; Laboratory Personnel; Magnetic Resonance Imaging; Methionine; Mice; Mice, Transgenic; Occupational Exposure; Occupational Injuries; Prion Proteins; Wounds, Stab

2020
System degeneration in an MM1-type sporadic Creutzfeldt-Jakob disease case with an unusually prolonged akinetic mutism state.
    Prion, 2021, Volume: 15, Issue:1

    Topics: Akinetic Mutism; Creutzfeldt-Jakob Syndrome; Humans; Male; Methionine; Middle Aged; Neurodegenerative Diseases; Prion Proteins

2021
Genetic Creutzfeldt-Jakob disease-M232R with the cooccurrence of multiple prion strains, M1 + M2C + M2T: Report of an autopsy case.
    Neuropathology : official journal of the Japanese Society of Neuropathology, 2021, Volume: 41, Issue:3

    Topics: Atrophy; Autopsy; Blotting, Western; Cerebellum; Cerebrum; Creutzfeldt-Jakob Syndrome; Humans; Japan; Magnetic Resonance Imaging; Male; Methionine; Middle Aged; Mutation; Myocardium; PrPSc Proteins; Thalamus

2021
Pathological progression of genetic Creutzfeldt-Jakob disease with a PrP V180I mutation.
    Prion, 2018, 01-02, Volume: 12, Issue:1

    Topics: Aged; Aged, 80 and over; Brain; Creutzfeldt-Jakob Syndrome; Disease Progression; Gliosis; Glutamic Acid; Humans; Isoleucine; Methionine; Middle Aged; Mutation; Prion Proteins; Vacuoles; Valine

2018
Rapid amplification of prions from variant Creutzfeldt-Jakob disease cerebrospinal fluid.
    The journal of pathology. Clinical research, 2018, Volume: 4, Issue:2

    Topics: Animals; Brain; Cattle; Codon; Creutzfeldt-Jakob Syndrome; Encephalopathy, Bovine Spongiform; Homozygote; Humans; Methionine; Prion Diseases; Prion Proteins; Retrospective Studies; Sensitivity and Specificity; tau Proteins; United Kingdom; Zoonoses

2018
Diagnosis of Methionine/Valine Variant Creutzfeldt-Jakob Disease by Protein Misfolding Cyclic Amplification.
    Emerging infectious diseases, 2018, Volume: 24, Issue:7

    Topics: Creutzfeldt-Jakob Syndrome; Genotype; Humans; Methionine; Prion Proteins; Proteostasis Deficiencies; Sensitivity and Specificity; Valine

2018
A case of V180I genetic Creutzfeldt-Jakob disease presenting with conspicuous facial mimicry.
    Prion, 2019, Volume: 13, Issue:1

    Topics: Aged, 80 and over; Codon; Creutzfeldt-Jakob Syndrome; Face; Female; Homozygote; Humans; Methionine; Point Mutation; Prion Proteins

2019
Association of prion protein genotype and scrapie prion protein type with cellular prion protein charge isoform profiles in cerebrospinal fluid of humans with sporadic or familial prion diseases.
    Neurobiology of aging, 2014, Volume: 35, Issue:5

    Topics: Adult; Aged; Aged, 80 and over; Codon; Creutzfeldt-Jakob Syndrome; Female; Genotype; Humans; Immunoblotting; Insomnia, Fatal Familial; Male; Methionine; Middle Aged; Mutation; Phosphorylation; Polymorphism, Genetic; Prion Diseases; Prion Proteins; Prions; Protein Isoforms; Protein Processing, Post-Translational; PrPC Proteins; PrPSc Proteins; Valine

2014
Familial Creutzfeldt-Jakob disease with M232R mutation presented with corticobasal syndrome.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2015, Volume: 36, Issue:7

    Topics: 14-3-3 Proteins; Aged; Arginine; Basal Ganglia; Brain Diseases; Cerebral Cortex; Creutzfeldt-Jakob Syndrome; DNA Mutational Analysis; Female; Humans; Methionine; Mutation; Prions

2015
Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to disease etiology?
    Journal of virology, 2015, Volume: 89, Issue:7

    Topics: Aged; Animals; Creutzfeldt-Jakob Syndrome; Disease Models, Animal; Female; Homozygote; Humans; Iatrogenic Disease; Male; Methionine; Mice; Middle Aged; Mutant Proteins; Mutation, Missense; Neurosurgical Procedures; Occupational Exposure; Prion Proteins; Prions; Surgeons

2015
Atypical sporadic CJD-MM phenotype with white matter kuru plaques associated with intranuclear inclusion body and argyrophilic grain disease.
    Neuropathology : official journal of the Japanese Society of Neuropathology, 2015, Volume: 35, Issue:4

    Topics: Aged; Brain; Creutzfeldt-Jakob Syndrome; Encephalopathy, Bovine Spongiform; Humans; Intranuclear Inclusion Bodies; Kuru; Male; Methionine; Phenotype; Tauopathies; White Matter

2015
Sporadic Creutzfeldt-Jakob Disease MM1+2C and MM1 are Identical in Transmission Properties.
    Brain pathology (Zurich, Switzerland), 2016, Volume: 26, Issue:1

    Topics: Aged; Animals; Brain; Creutzfeldt-Jakob Syndrome; Disease Models, Animal; Female; Genotype; Humans; Male; Methionine; Mice; Mutant Proteins; Prion Proteins; Prions

2016
Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years.
    Brain : a journal of neurology, 2015, Volume: 138, Issue:Pt 11

    Topics: Adult; Brain; Codon; Creutzfeldt-Jakob Syndrome; Disease Progression; Drug Contamination; Electroencephalography; Female; Gene-Environment Interaction; Genotype; Growth Disorders; Homozygote; Human Growth Hormone; Humans; Iatrogenic Disease; Infectious Disease Incubation Period; Magnetic Resonance Imaging; Male; Methionine; Middle Aged; Prion Proteins; Prions; Retrospective Studies; Time Factors; United Kingdom; Valine

2015
Roles of methionine oxidation in E200K prion protein misfolding: Implications for the mechanism of pathogenesis in E200K linked familial Creutzfeldt-Jakob disease.
    Biochimica et biophysica acta, 2016, Volume: 1864, Issue:4

    Topics: Creutzfeldt-Jakob Syndrome; Humans; Hydrogen-Ion Concentration; Methionine; Oxidation-Reduction; Prions; Protein Aggregates; Protein Folding; Protein Multimerization

2016
The Distribution of Prion Protein Allotypes Differs Between Sporadic and Iatrogenic Creutzfeldt-Jakob Disease Patients.
    PLoS pathogens, 2016, Volume: 12, Issue:2

    Topics: Adult; Aged; Brain; Brain Chemistry; Creutzfeldt-Jakob Syndrome; Female; Humans; Iatrogenic Disease; Male; Methionine; Middle Aged; Phenotype; PrPC Proteins; PrPSc Proteins; Recombinant Proteins; Valine

2016
Distinctive properties of plaque-type dura mater graft-associated Creutzfeldt-Jakob disease in cell-protein misfolding cyclic amplification.
    Laboratory investigation; a journal of technical methods and pathology, 2016, Volume: 96, Issue:5

    Topics: Brain; Codon; Creutzfeldt-Jakob Syndrome; Dura Mater; Homozygote; Humans; Methionine; Prion Proteins; Protein Folding; PrPSc Proteins; Valine

2016
Prion Strain Characterization of a Novel Subtype of Creutzfeldt-Jakob Disease.
    Journal of virology, 2017, 06-01, Volume: 91, Issue:11

    Topics: Animals; Arvicolinae; Brain; Brain Chemistry; Creutzfeldt-Jakob Syndrome; Genotype; Humans; Methionine; Mice; Mice, Transgenic; Phenotype; Prion Proteins; Prions; Protein Conformation; Valine

2017
Population distribution of the methionine allele at the PRNP codon 129 polymorphism in Europe and the Middle East.
    Human biology, 2008, Volume: 80, Issue:2

    Topics: Alleles; Creutzfeldt-Jakob Syndrome; Europe; Gene Frequency; Genetics, Population; Humans; Methionine; Middle East; Polymorphism, Genetic; Prion Proteins; Prions; Risk Factors; Turkey

2008
Sleep disturbances in variant of Creutzfeldt-Jakob disease.
    Sleep medicine, 2009, Volume: 10, Issue:6

    Topics: Adult; Codon; Creutzfeldt-Jakob Syndrome; Female; Homozygote; Humans; Methionine; Polymorphism, Genetic; Polysomnography; Prions; Sleep Initiation and Maintenance Disorders

2009
Prevalence of variant CJD in the UK.
    BMJ (Clinical research ed.), 2009, May-21, Volume: 338

    Topics: Creutzfeldt-Jakob Syndrome; Heterozygote; Homozygote; Humans; Methionine; Prevalence; United Kingdom; Valine

2009
Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification.
    Acta neuropathologica, 2009, Volume: 118, Issue:5

    Topics: Aged; Aged, 80 and over; Brain; Creutzfeldt-Jakob Syndrome; DNA Mutational Analysis; Female; Humans; Incidence; Male; Methionine; Middle Aged; Neurologic Examination; Phenotype; Plaque, Amyloid; PrPSc Proteins; Retrospective Studies; Valine

2009
Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics.
    Brain : a journal of neurology, 2009, Volume: 132, Issue:Pt 10

    Topics: Aged; Antibody Specificity; Autopsy; Blotting, Western; Brain Chemistry; Creutzfeldt-Jakob Syndrome; DNA; Endopeptidase K; Female; Humans; Immunoassay; Immunohistochemistry; Indicators and Reagents; Male; Methionine; Middle Aged; Phenotype; Prions; Protein Conformation; Valine

2009
Rare E196K mutation in the PRNP gene of a patient exhibiting behavioral abnormalities.
    Clinical neurology and neurosurgery, 2010, Volume: 112, Issue:3

    Topics: 14-3-3 Proteins; Aged, 80 and over; Brain; Codon; Creutzfeldt-Jakob Syndrome; Electroencephalography; Genetic Predisposition to Disease; Homozygote; Humans; Magnetic Resonance Imaging; Male; Methionine; Mutation; Myoclonus; Polymorphism, Single Nucleotide; Prion Proteins; Prions

2010
Elevated phosphorylated tau pT-181 in a possible PRNP codon 129 MV vCJD case.
    Journal of neurology, neurosurgery, and psychiatry, 2010, Volume: 81, Issue:12

    Topics: 14-3-3 Proteins; Adult; Amino Acid Substitution; Blotting, Western; Brain; Codon; Creutzfeldt-Jakob Syndrome; Dementia; Enzyme-Linked Immunosorbent Assay; Gene Expression; Genetic Carrier Screening; Humans; Magnetic Resonance Imaging; Male; Methionine; Nerve Growth Factors; Phosphorylation; Prion Proteins; Prions; Pulvinar; S100 Calcium Binding Protein beta Subunit; S100 Proteins; tau Proteins; Threonine; Valine

2010
Experience with preventive genetic testing of corneal donors in slovakia.
    Cornea, 2011, Volume: 30, Issue:9

    Topics: Age Distribution; Aged; Codon; Cornea; Corneal Transplantation; Creutzfeldt-Jakob Syndrome; Disease Transmission, Infectious; Genetic Testing; Genotype; Humans; Methionine; Middle Aged; Point Mutation; Polymerase Chain Reaction; Prion Proteins; Prions; Slovakia; Tissue Donors; Valine

2011
Slow-progressive ataxia with a methionine-to-arginine point mutation in codon 232 in the prion protein gene (PRNP).
    Clinical neurology and neurosurgery, 2011, Volume: 113, Issue:8

    Topics: Arginine; Cerebellar Ataxia; Cerebral Angiography; Creutzfeldt-Jakob Syndrome; Electroencephalography; Female; Humans; Magnetic Resonance Angiography; Magnetic Resonance Imaging; Methionine; Middle Aged; Point Mutation; Prion Proteins; Prions; Tomography, Emission-Computed, Single-Photon; Vision Disorders

2011
Leu138 in bovine prion peptide fibrils is involved in seeding discrimination related to codon 129 M/V polymorphism in the prion peptide seeding experiment.
    The FEBS journal, 2011, Volume: 278, Issue:22

    Topics: Amyloid; Animals; Cattle; Circular Dichroism; Codon; Creutzfeldt-Jakob Syndrome; Humans; Leucine; Methionine; Peptide Fragments; Polymorphism, Genetic; Prions; Rats; Spectroscopy, Fourier Transform Infrared

2011
Protease-sensitive conformers in broad spectrum of distinct PrPSc structures in sporadic Creutzfeldt-Jakob disease are indicator of progression rate.
    PLoS pathogens, 2011, Volume: 7, Issue:9

    Topics: Aged; Aged, 80 and over; Brain; Codon; Creutzfeldt-Jakob Syndrome; Disease Progression; Epitopes; Female; Homozygote; Humans; Male; Methionine; Middle Aged; Nonlinear Dynamics; Peptide Hydrolases; Phenotype; Prion Proteins; Prions; PrPSc Proteins; Regression Analysis; Valine

2011
Comparison of the pathologic and pathogenic features in six different regions of postmortem brains of three patients with fatal familial insomnia.
    International journal of molecular medicine, 2013, Volume: 31, Issue:1

    Topics: Adult; Animals; Autopsy; Blotting, Western; Chromosomes, Human, Pair 20; Codon; Creutzfeldt-Jakob Syndrome; Endopeptidase K; Female; Glial Fibrillary Acidic Protein; Gyrus Cinguli; Humans; Immunohistochemistry; Insomnia, Fatal Familial; Male; Methionine; Mice; Mice, Inbred C57BL; Middle Aged; Mutation; Pedigree; Phosphopyruvate Hydratase; Prefrontal Cortex; Prion Proteins; Prions; Real-Time Polymerase Chain Reaction; RNA, Messenger; Specimen Handling; Thalamus; Transcription Factors

2013
Creutzfeldt-Jakob disease with E200K mutation in Slovakia: characterization and development.
    Acta virologica, 2002, Volume: 46, Issue:1

    Topics: Amino Acid Substitution; Creutzfeldt-Jakob Syndrome; Female; Genotype; Humans; Male; Methionine; Mutation; Pedigree; Phenotype; Prions; Restriction Mapping; Risk Factors; Slovakia; Valine

2002
A French cluster of Creutzfeldt-Jakob disease: a molecular analysis.
    European journal of neurology, 2002, Volume: 9, Issue:5

    Topics: Aged; Brain; Cluster Analysis; Codon; Creutzfeldt-Jakob Syndrome; DNA Mutational Analysis; Female; France; Genetic Testing; Genotype; Humans; Male; Methionine; Middle Aged; Mutation; Polymorphism, Genetic; PrPSc Proteins

2002
Distinctive cerebellar immunoreactivity for the prion protein in familial (E200K) Creutzfeldt-Jakob disease.
    Acta neuropathologica, 2003, Volume: 105, Issue:5

    Topics: Cerebellum; Creutzfeldt-Jakob Syndrome; Genetic Variation; Glutamic Acid; Humans; Immunohistochemistry; Lysine; Methionine; Mutation; Prions; Valine

2003
Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics.
    Science (New York, N.Y.), 2003, Apr-25, Volume: 300, Issue:5619

    Topics: Adult; Animals; Cannibalism; Child; Codon; Creutzfeldt-Jakob Syndrome; Disease Outbreaks; Ethnicity; Female; Gene Frequency; Haplotypes; Heterozygote; History, 19th Century; History, 20th Century; History, Ancient; Homozygote; Humans; Immunity, Innate; Kuru; Linkage Disequilibrium; Male; Methionine; Middle Aged; Mutation; Papua New Guinea; Polymorphism, Genetic; PrPC Proteins; Selection, Genetic; Valine

2003
Future uncertain for variant Creutzfeldt-Jakob disease.
    The Lancet. Neurology, 2002, Volume: 1, Issue:5

    Topics: Creutzfeldt-Jakob Syndrome; Homozygote; Humans; Methionine; Mutation; Prions; United Kingdom

2002
The residue 129 polymorphism in human prion protein does not confer susceptibility to Creutzfeldt-Jakob disease by altering the structure or global stability of PrPC.
    The Journal of biological chemistry, 2004, Jul-02, Volume: 279, Issue:27

    Topics: Amides; Circular Dichroism; Creutzfeldt-Jakob Syndrome; Escherichia coli; Genetic Predisposition to Disease; Humans; Kinetics; Magnetic Resonance Spectroscopy; Methionine; Models, Molecular; Mutation; Plasmids; Polymorphism, Genetic; Protein Conformation; Protein Folding; Protein Structure, Secondary; Protein Structure, Tertiary; PrPC Proteins; Time Factors; Valine

2004
Methionine 129 variant of human prion protein oligomerizes more rapidly than the valine 129 variant: implications for disease susceptibility to Creutzfeldt-Jakob disease.
    The Journal of biological chemistry, 2004, Jul-23, Volume: 279, Issue:30

    Topics: Alleles; Amino Acid Sequence; Creutzfeldt-Jakob Syndrome; Endopeptidase K; Genetic Variation; Heterozygote; Homozygote; Humans; In Vitro Techniques; Methionine; Models, Molecular; Molecular Sequence Data; Prions; Protein Folding; Protein Structure, Quaternary; Protein Structure, Secondary; Recombinant Proteins; Valine

2004
Expression of excitatory amino acid transporter-1 (EAAT-1) in brain macrophages and microglia of patients with prion diseases.
    Journal of neuropathology and experimental neurology, 2004, Volume: 63, Issue:10

    Topics: Adult; Aged; Amyloid; Brain; Case-Control Studies; Child; Codon; Creutzfeldt-Jakob Syndrome; Excitatory Amino Acid Transporter 1; Female; Heterozygote; Humans; Insomnia, Fatal Familial; Macrophages; Male; Methionine; Microglia; Middle Aged; Prion Proteins; Prions; Protein Precursors; Severity of Illness Index; Time Factors; Valine

2004
Human prion protein with valine 129 prevents expression of variant CJD phenotype.
    Science (New York, N.Y.), 2004, Dec-03, Volume: 306, Issue:5702

    Topics: Amyloid; Animals; Brain; Cattle; Creutzfeldt-Jakob Syndrome; Encephalopathy, Bovine Spongiform; Humans; Methionine; Mice; Mice, Transgenic; Phenotype; Polymorphism, Genetic; Prion Proteins; Prions; Protein Conformation; Protein Precursors; PrPC Proteins; PrPSc Proteins; Valine

2004
Biomedicine. Prion dormancy and disease.
    Science (New York, N.Y.), 2004, Dec-03, Volume: 306, Issue:5702

    Topics: Animals; Appendix; Brain; Carrier State; Cattle; Creutzfeldt-Jakob Syndrome; Disease Outbreaks; Encephalopathy, Bovine Spongiform; Genetic Predisposition to Disease; Genotype; Humans; Methionine; Mice; Mice, Transgenic; Polymorphism, Genetic; Protein Conformation; PrPC Proteins; United Kingdom; Valine

2004
Type 1 and type 2 human PrPSc have different aggregation sizes in methionine homozygotes with sporadic, iatrogenic and variant Creutzfeldt-Jakob disease.
    The Journal of general virology, 2005, Volume: 86, Issue:Pt 1

    Topics: Blotting, Western; Brain Chemistry; Creutzfeldt-Jakob Syndrome; Filtration; Homozygote; Humans; Iatrogenic Disease; Methionine; Plaque, Amyloid; PrPSc Proteins

2005
Genetic susceptibility to prion disease: new phenotypes?
    The Lancet. Neurology, 2005, Volume: 4, Issue:1

    Topics: Animals; Cattle; Creutzfeldt-Jakob Syndrome; Encephalopathy, Bovine Spongiform; Genetic Predisposition to Disease; Homozygote; Humans; Methionine; Phenotype; Prion Diseases

2005
Creutzfeldt-Jakob disease associated with the R208H mutation in the prion protein gene.
    Neurology, 2005, Mar-08, Volume: 64, Issue:5

    Topics: 14-3-3 Proteins; Amino Acid Substitution; Brain; Creutzfeldt-Jakob Syndrome; Disease Progression; DNA Mutational Analysis; Fatal Outcome; Female; Genetic Predisposition to Disease; Genotype; Homozygote; Humans; Immunoblotting; Immunohistochemistry; Mass Spectrometry; Methionine; Middle Aged; Mutation; Neurons; Phenotype; PrPSc Proteins

2005
Creutzfeldt-Jakob disease (CJD) with a mutation at codon 148 of prion protein gene: relationship with sporadic CJD.
    The American journal of pathology, 2005, Volume: 167, Issue:6

    Topics: Amino Acid Substitution; Autopsy; Brain; Codon; Creutzfeldt-Jakob Syndrome; Female; Genetic Carrier Screening; Genotype; Humans; Male; Methionine; Middle Aged; Mutation; Open Reading Frames; Pedigree; Phenotype; Protein Conformation; PrPC Proteins; PrPSc Proteins; Valine

2005
Analysis of PRNP gene codon 129 polymorphism in the Greek population.
    European journal of epidemiology, 2006, Volume: 21, Issue:3

    Topics: Adult; Aged; Codon; Creutzfeldt-Jakob Syndrome; Female; Gene Frequency; Genetics, Population; Genotype; Greece; Humans; Male; Methionine; Middle Aged; Polymerase Chain Reaction; Polymorphism, Genetic; Prion Proteins; Prions; Valine; White People

2006
Long-duration sCJD with PRNP codon 129 methionine homozygosity and cerebral cortical plaques.
    Neurology, 2006, Jun-27, Volume: 66, Issue:12

    Topics: Adult; Amyloid; Brain Diseases; Codon; Creutzfeldt-Jakob Syndrome; Female; Genetic Predisposition to Disease; Homozygote; Humans; Kuru; Methionine; Mutation; Plaque, Amyloid; Prion Proteins; Prions; Protein Precursors; Time Factors

2006
Can a second wave of new variant of the CJD be discarded in absence of observation of clinical non Met-Met cases?
    Revue d'epidemiologie et de sante publique, 2006, Volume: 54, Issue:2

    Topics: Creutzfeldt-Jakob Syndrome; Humans; Methionine; United Kingdom

2006
Autopsy-proven Creutzfeldt-Jakob disease with a codon 180 mutation showing dissociation between diffusion-weighted magnetic resonance imaging and single-photon emission computed tomography findings: Is this a suggestive finding in long survival?
    European neurology, 2006, Volume: 56, Issue:1

    Topics: Aged; Autopsy; Cerebral Cortex; Creutzfeldt-Jakob Syndrome; Diffusion Magnetic Resonance Imaging; Female; Humans; Isoleucine; Methionine; Mutation; Prions; Tomography, Emission-Computed, Single-Photon; Valine

2006
Autopsy case of Creutzfeldt-Jakob disease with Met/Val heterozygosity at codon 129 and type 1 protease-resistant prion protein presenting some florid-type plaques and many Kuru plaques in the cerebellum.
    Neuropathology : official journal of the Japanese Society of Neuropathology, 2006, Volume: 26, Issue:4

    Topics: Autopsy; Blotting, Western; Cerebellum; Creutzfeldt-Jakob Syndrome; Female; Genotype; Humans; Kuru; Methionine; Middle Aged; Peptide Hydrolases; Plaque, Amyloid; Polymerase Chain Reaction; Polymorphism, Genetic; Prions; Valine

2006
Regulatory sequences of the PRNP gene influence susceptibility to sporadic Creutzfeldt-Jakob disease.
    Neuroscience letters, 2007, Jan-16, Volume: 411, Issue:3

    Topics: Creutzfeldt-Jakob Syndrome; Female; Gene Frequency; Genetic Predisposition to Disease; Genotype; Humans; Male; Methionine; Polymorphism, Single Nucleotide; Prion Proteins; Prions; Regulatory Elements, Transcriptional; Valine

2007
Variant Creutzfeldt-Jakob [corrected] disease: the second case in Portugal and in the same geographical region.
    Journal of neurology, neurosurgery, and psychiatry, 2008, Volume: 79, Issue:2

    Topics: Adolescent; Brain; Creutzfeldt-Jakob Syndrome; Diagnosis, Differential; Diffusion Magnetic Resonance Imaging; Disease Outbreaks; Disease Progression; Fatal Outcome; Female; Homozygote; Humans; Methionine; Neurologic Examination; Neuropsychological Tests; Topography, Medical

2008
Two different clinical phenotypes of Creutzfeldt-Jakob disease with a M232R substitution.
    Journal of neurology, 2007, Volume: 254, Issue:11

    Topics: 14-3-3 Proteins; Aged; Arginine; Creutzfeldt-Jakob Syndrome; Electroencephalography; Female; Humans; Magnetic Resonance Imaging; Male; Methionine; Middle Aged; Mutation; Phenotype; Prions

2007
A novel phenotype of sporadic Creutzfeldt-Jakob disease.
    Journal of neurology, neurosurgery, and psychiatry, 2007, Volume: 78, Issue:12

    Topics: Aged; Antibodies; Antibodies, Monoclonal; Antiparkinson Agents; Blotting, Western; Brain; Codon; Creutzfeldt-Jakob Syndrome; Female; Humans; Immunohistochemistry; Levodopa; Magnetic Resonance Imaging; Methionine; Parkinsonian Disorders; Phenotype; Polymorphism, Genetic; PrPSc Proteins; Valine

2007
Differences of apparent diffusion coefficient values in patients with Creutzfeldt-Jakob disease according to the codon 129 genotype.
    AJNR. American journal of neuroradiology, 2008, Volume: 29, Issue:7

    Topics: Basal Ganglia; Caudate Nucleus; Cerebral Cortex; Codon; Corpus Striatum; Creutzfeldt-Jakob Syndrome; Diffusion Magnetic Resonance Imaging; Genotype; Humans; Methionine; Polymorphism, Genetic; Prions; Pulvinar; Retrospective Studies; Valine

2008
Neuropathological phenotype and 'prion protein' genotype correlation in sporadic Creutzfeldt-Jakob disease.
    Neuroscience letters, 1994, Sep-26, Volume: 179, Issue:1-2

    Topics: Aged; Aged, 80 and over; Amyloid; Amyloidosis; Creutzfeldt-Jakob Syndrome; DNA; Genotype; Humans; Methionine; Middle Aged; Open Reading Frames; Phenotype; Polymerase Chain Reaction; Polymorphism, Genetic; Prions; Valine

1994
Iatrogenic Creutzfeldt-Jakob disease: an example of the interplay between ancient genes and modern medicine.
    Neurology, 1994, Volume: 44, Issue:2

    Topics: Amyloid; Base Sequence; Brain; Chromosomes, Human, Pair 20; Codon; Corneal Transplantation; Creutzfeldt-Jakob Syndrome; Deoxyribonucleases, Type II Site-Specific; DNA; DNA Primers; Dura Mater; Electroencephalography; Genotype; Gonadotropins; Growth Hormone; Homozygote; Humans; Iatrogenic Disease; Methionine; Molecular Sequence Data; Open Reading Frames; Point Mutation; Restriction Mapping; Transplantation, Homologous; Valine

1994
Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease.
    Annals of neurology, 1996, Volume: 39, Issue:6

    Topics: Adult; Aged; Astrocytes; Blotting, Western; Brain; Codon; Creutzfeldt-Jakob Syndrome; Culture Techniques; Endopeptidase K; Female; Genotype; Glycosylation; Homozygote; Humans; Immunohistochemistry; Male; Methionine; Middle Aged; Phenotype; Prions; Serine Endopeptidases

1996
Putative neurosurgical transmission of Creutzfeldt-Jakob disease with analysis of donor and recipient: agent strains.
    Comptes rendus de l'Academie des sciences. Serie III, Sciences de la vie, 1997, Volume: 320, Issue:4

    Topics: Biopsy; Brain; Creutzfeldt-Jakob Syndrome; Female; Genotype; Humans; Iatrogenic Disease; Immunohistochemistry; Male; Methionine; Middle Aged; Neurosurgery; Phenotype; Prions; Valine

1997
Phenotype-genotype studies in kuru: implications for new variant Creutzfeldt-Jakob disease.
    Proceedings of the National Academy of Sciences of the United States of America, 1998, Oct-27, Volume: 95, Issue:22

    Topics: Adolescent; Adult; Age of Onset; Amyloid; Child; Chromosomes, Human, Pair 20; Codon; Creutzfeldt-Jakob Syndrome; DNA; Female; Genetic Variation; Genotype; Humans; Kuru; Male; Methionine; Middle Aged; Papua New Guinea; Phenotype; Point Mutation; Prion Proteins; Prions; Protein Precursors; Retrospective Studies; Valine

1998
Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease.
    Lancet (London, England), 1999, May-15, Volume: 353, Issue:9165

    Topics: Aged; Aged, 80 and over; Codon; Creutzfeldt-Jakob Syndrome; Genotype; Homozygote; Humans; Methionine; Middle Aged; Molecular Epidemiology; Prions; Valine

1999
Diagnosis of new variant Creutzfeldt-Jakob disease.
    Annals of neurology, 2000, Volume: 47, Issue:5

    Topics: Adolescent; Adult; Ataxia; Brain; Codon; Cognition Disorders; Creutzfeldt-Jakob Syndrome; Diagnosis, Differential; Dyskinesias; Female; Homozygote; Humans; Magnetic Resonance Imaging; Male; Mental Disorders; Methionine; Middle Aged; Neuropsychological Tests; Population Surveillance; Prions; Sensitivity and Specificity; United Kingdom

2000
Trends in mortality from sporadic Creutzfeldt-Jakob disease in France 1992-7.
    Journal of neurology, neurosurgery, and psychiatry, 2000, Volume: 68, Issue:6

    Topics: Aged; Aged, 80 and over; Cause of Death; Codon; Creutzfeldt-Jakob Syndrome; Cross-Sectional Studies; France; Genotype; Humans; Incidence; Methionine; Middle Aged; Prions; Valine

2000
Predictability of the UK variant Creutzfeldt-Jakob disease epidemic.
    Science (New York, N.Y.), 2001, Nov-23, Volume: 294, Issue:5547

    Topics: Adolescent; Adult; Age Distribution; Age Factors; Age of Onset; Animals; Cattle; Child; Child, Preschool; Creutzfeldt-Jakob Syndrome; Diet; Disease Susceptibility; Encephalopathy, Bovine Spongiform; Genetic Variation; Genotype; Humans; Incidence; Infant; Likelihood Functions; Methionine; Mice; Models, Biological; Prevalence; Prions; United Kingdom

2001