Co-existence of PrP

Adult Aged Aged, 80 and over Brain / metabolism Cohort Studies Creutzfeldt-Jakob Syndrome / genetics Female Genetic Association Studies Humans Male Middle Aged PrPSc Proteins / chemistry Protein Conformation Protein Stability Tissue Distribution Valine / chemistry

Journal

Scientific reports
ISSN: 2045-2322
Titre abrégé: Sci Rep
Pays: England
ID NLM: 101563288

Informations de publication

Date de publication:
30 01 2020
Historique:
received: 10 07 2019
accepted: 28 12 2019
entrez: 1 2 2020
pubmed: 1 2 2020
medline: 18 11 2020
Statut: epublish

Résumé

We report a detailed study of a cohort of sporadic Creutzfeldt-Jakob disease (sCJD) VV1-2 type-mixed cases (valine homozygosity at codon 129 of the prion protein, PrP, gene harboring disease-related PrP, PrP

Identifiants

DOI: 10.1038/s41598-020-58446-0 PMID: 32001774 PMC: PMC6992672
pubmed: 32001774
doi: 10.1038/s41598-020-58446-0
pii: 10.1038/s41598-020-58446-0
pmc: PMC6992672
doi:

Substances chimiques

PrPSc Proteins 0
Valine HG18B9YRS7

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1503

Subventions

Organisme : NIDDK NIH HHS
ID : K01 DK107787
Pays : United States
Organisme : NIA NIH HHS
ID : P30 AG062428
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS083687
Pays : United States
Organisme : NIAID NIH HHS
ID : P01 AI077774
Pays : United States
Organisme : NIAID NIH HHS
ID : P01 AI106705
Pays : United States

Références

Parchi, P. et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann. Neurol. 46, 224–233 (1999).
doi: 10.1002/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W
Puoti, G. et al. Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brain. Neurology 53, 2173–2176, https://doi.org/10.1212/wnl.53.9.2173 (1999).
doi: 10.1212/wnl.53.9.2173 pubmed: 10599800
Polymenidou, M. et al. Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease. Lancet Neurol. 4, 805–814, https://doi.org/10.1016/S1474-4422(05)70225-8 (2005).
doi: 10.1016/S1474-4422(05)70225-8 pubmed: 16297838
Cali, I. et al. 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 J. Neurol. 132, 2643–2658, https://doi.org/10.1093/brain/awp196 (2009).
doi: 10.1093/brain/awp196
Notari, S. et al. A refined method for molecular typing reveals that co-occurrence of PrP(Sc) types in Creutzfeldt-Jakob disease is not the rule. Lab. Investig. J. Tech. Methods Pathol. 87, 1103–1112, https://doi.org/10.1038/labinvest.3700676 (2007).
doi: 10.1038/labinvest.3700676
Parchi, P. et al. Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification. Acta Neuropathol. (Berl.) 118, 659–671, https://doi.org/10.1007/s00401-009-0585-1 (2009).
doi: 10.1007/s00401-009-0585-1
Cronier, S., Beringue, V., Bellon, A., Peyrin, J.-M. & Laude, H. Prion strain- and species-dependent effects of antiprion molecules in primary neuronal cultures. J. Virol. 81, 13794–13800, https://doi.org/10.1128/JVI.01502-07 (2007).
doi: 10.1128/JVI.01502-07 pubmed: 17913812 pmcid: 2168876
Foutz, A. et al. Diagnostic and prognostic value of human prion detection in cerebrospinal fluid. Ann. Neurol. 81, 79–92, https://doi.org/10.1002/ana.24833 (2017).
doi: 10.1002/ana.24833 pubmed: 27893164 pmcid: 5266667
Giles, K., Olson, S. H. & Prusiner, S. B. Developing Therapeutics for PrP Prion Diseases. Cold Spring Harb. Perspect. Med. 7, https://doi.org/10.1101/cshperspect.a023747 (2017).
doi: 10.1101/cshperspect.a023747
Parchi, P. et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann. Neurol. 39, 767–778, https://doi.org/10.1002/ana.410390613 (1996).
doi: 10.1002/ana.410390613 pubmed: 8651649
Notari, S. et al. Effects of different experimental conditions on the PrPSc core generated by protease digestion: implications for strain typing and molecular classification of CJD. J. Biol. Chem. 279, 16797–16804, https://doi.org/10.1074/jbc.M313220200 (2004).
doi: 10.1074/jbc.M313220200 pubmed: 14754888
Parchi, P., Capellari, S. & Gambetti, P. Intracerebral distribution of the abnormal isoform of the prion protein in sporadic Creutzfeldt-Jakob disease and fatal insomnia. Microsc. Res. Tech. 50, 16–25, 10.1002/1097-0029(20000701)50:1<16::AID-JEMT4>3.0.CO;2-Y (2000).
Schoch, G. et al. Analysis of prion strains by PrPSc profiling in sporadic Creutzfeldt-Jakob disease. PLoS Med. 3, e14, https://doi.org/10.1371/journal.pmed.0030014 (2006).
doi: 10.1371/journal.pmed.0030014 pubmed: 16354106
Bishop, M. T., Will, R. G. & Manson, J. C. Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc. Natl. Acad. Sci. USA 107, 12005–12010, https://doi.org/10.1073/pnas.1004688107 (2010).
doi: 10.1073/pnas.1004688107 pubmed: 20547859
Gambetti, P. et al. Molecular biology and pathology of prion strains in sporadic human prion diseases. Acta Neuropathol. (Berl.) 121, 79–90, https://doi.org/10.1007/s00401-010-0761-3 (2011).
doi: 10.1007/s00401-010-0761-3
Kobayashi, A. et al. Deciphering the pathogenesis of sporadic Creutzfeldt-Jakob disease with codon 129 M/V and type 2 abnormal prion protein. Acta Neuropathol. Commun. 1, 74, https://doi.org/10.1186/2051-5960-1-74 (2013).
doi: 10.1186/2051-5960-1-74 pubmed: 24252157 pmcid: 3833290
Parchi, P. et al. Genetic influence on the structural variations of the abnormal prion protein. Proc. Natl. Acad. Sci. USA 97, 10168–10172, https://doi.org/10.1073/pnas.97.18.10168 (2000).
doi: 10.1073/pnas.97.18.10168 pubmed: 10963679
Cali, I. et al. Classification of sporadic Creutzfeldt-Jakob disease revisited. Brain J. Neurol. 129, 2266–2277, https://doi.org/10.1093/brain/awl224 (2006).
doi: 10.1093/brain/awl224
Nemani, S. K. et al. Co-occurrence of chronic traumatic encephalopathy and prion disease. Acta Neuropathol. Commun. 6, 140, https://doi.org/10.1186/s40478-018-0643-9 (2018).
doi: 10.1186/s40478-018-0643-9 pubmed: 30563563 pmcid: 6299534
Kobayashi, A. et al. Experimental verification of a traceback phenomenon in prion infection. J. Virol. 84, 3230–3238, https://doi.org/10.1128/JVI.02387-09 (2010).
doi: 10.1128/JVI.02387-09 pubmed: 20089646 pmcid: 2838106

Auteurs

Ignazio Cali (I)

Department of Pathology, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA.
National Prion Disease Pathology Surveillance Center, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA.

Gianfranco Puoti (G)

Department of Advanced Medical and Surgical Sciences, University of Campania "L. Vanvitelli", Caserta, 81100, Italy.

Jason Smucny (J)

Department of Psychiatry, University of California, Davis, CA, 95616, USA.

Paul Michael Curtiss (PM)

New York University, School of Medicine, New York, NY, 10016, USA.

Laura Cracco (L)

Department of Pathology, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA.

Tetsuyuki Kitamoto (T)

Department of Neurological Science, Tohoku University Graduate School of Medicine, Sendai, 980-8576, Japan.

Rossana Occhipinti (R)

Department of Physiology and Biophysics, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA.

Mark Lloyd Cohen (ML)

Department of Pathology, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA.
National Prion Disease Pathology Surveillance Center, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA.

Brian Stephen Appleby (BS)

Department of Neurology, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA.
Department of Psychiatry, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA.
National Prion Disease Pathology Surveillance Center, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA.

Pierluigi Gambetti (P)

Department of Pathology, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA. pxg13@case.edu.

Articles similaires

[Redispensing of expensive oral anticancer medicines: a practical application].

Lisanne N van Merendonk, Kübra Akgöl, Bastiaan Nuijen
1.00
Humans Antineoplastic Agents Administration, Oral Drug Costs Counterfeit Drugs

Smoking Cessation and Incident Cardiovascular Disease.

Jun Hwan Cho, Seung Yong Shin, Hoseob Kim et al.
1.00
Humans Male Smoking Cessation Cardiovascular Diseases Female

Evaluation of Low-Value Services Across Major Medicare Advantage Insurers and Traditional Medicare.

Ciara Duggan, Adam L Beckman, Ishani Ganguli et al.
1.00
Humans United States Aged Cross-Sectional Studies Medicare Part C

Effectiveness of Virtual Yoga for Chronic Low Back Pain: A Randomized Clinical Trial.

Hallie Tankha, Devyn Gaskins, Amanda Shallcross et al.
1.00
Humans Yoga Low Back Pain Female Male

Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Co-existence of PrP
questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Sujet âgé › Co-existence of PrP
questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Sujet âgé › Sujet âgé de 80 ans ou plus › Co-existence of PrP
questionsmedicales.fr › Système nerveux › Système nerveux central › Encéphale › Co-existence of PrP
questionsmedicales.fr › Environnement et santé publique › Santé publique › Méthodes épidémiologiques › Caractéristiques des études épidémiologiques › Études épidémiologiques › Études de cohortes › Co-existence of PrP
questionsmedicales.fr › Troubles mentaux › Troubles neurocognitifs › Démence › Maladie de Creutzfeldt-Jakob › Co-existence of PrP
questionsmedicales.fr › Techniques d'investigation › Techniques génétiques › Études d'associations génétiques › Co-existence of PrP
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Co-existence of PrP
questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Adulte d'âge moyen › Co-existence of PrP
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Prions › Protéines prion › Protéines PrPSc › Co-existence of PrP
questionsmedicales.fr › Phénomènes chimiques › Phénomènes biochimiques › Conformation moléculaire › Conformation des protéines › Co-existence of PrP
questionsmedicales.fr › Phénomènes chimiques › Phénomènes biochimiques › Stabilité protéique › Co-existence of PrP
questionsmedicales.fr › Phénomènes physiologiques › Phénomènes pharmacologiques et toxicologiques › Pharmacocinétique › Distribution tissulaire › Co-existence of PrP
questionsmedicales.fr › Acides aminés, peptides et protéines › Acides aminés › Acides aminés essentiels › Valine › Co-existence of PrP