Works matching Gerstmann-Sträussler-Scheinker syndrome (GSS)
Results: 30
The prion protein protease sensitivity, stability and seeding activity in variably protease sensitive prionopathy brain tissue suggests molecular overlaps with sporadic Creutzfeldt-Jakob disease.
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- Acta Neuropathologica Communications, 2014, v. 2, n. 1, p. 1, doi. 10.1186/s40478-014-0152-4
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Familial Prion Disease: First Indian Kindred with Gerstmann-Sträussler-Scheinker Syndrome.
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- 2020
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- journal article
Putative aggregation initiation sites in prion protein
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- FEBS Letters, 2006, v. 580, n. 8, p. 2033, doi. 10.1016/j.febslet.2006.03.002
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microRNA-146a-5p, Neurotropic Viral Infection and Prion Disease (PrD).
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- International Journal of Molecular Sciences, 2021, v. 22, n. 17, p. 9198, doi. 10.3390/ijms22179198
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Distribution of prion protein in German patients with Creutzfeldt-Jakob disease is different from that in Japanese patients.
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- Acta Neuropathologica, 1994, v. 87, n. 5, p. 481, doi. 10.1007/BF00294174
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A CONTRIBUIÇÃO GENÉTICA NO DESENVOLVIMENTO DE DOENÇAS PRIÔNICAS: REVISÃO SISTEMÁTICA.
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- Revista Foco (Interdisciplinary Studies Journal), 2023, v. 16, n. 6, p. 1, doi. 10.54751/revistafoco.v16n6-096
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Recent Advances in Our Molecular and Mechanistic Understanding of Misfolded Cellular Proteins in Alzheimer's Disease (AD) and Prion Disease (PrD).
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- Biomolecules (2218-273X), 2022, v. 12, n. 2, p. 166, doi. 10.3390/biom12020166
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Evaluation of Human Cerebrospinal Fluid Malate Dehydrogenase 1 as a Marker in Genetic Prion Disease Patients.
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- Biomolecules (2218-273X), 2019, v. 9, n. 12, p. 800, doi. 10.3390/biom9120800
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Scrapie-like prion protein accumulates in aggresomes of cyclosporin A-treated cells.
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- EMBO Journal, 2003, v. 22, n. 3, p. 404, doi. 10.1093/emboj/cdg045
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A family with hereditary cerebellar ataxia finally confirmed as Gerstmann-Staräfusser-Schreiber syndrome with P102L mutation in PRNP gene.
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- Neurosciences, 2017, v. 22, n. 2, p. 138, doi. 10.17712/nsj.2017.2.20160522
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Inherited Prion Disease A117V Is Not Simply a Proteinopathy but Produces Prions Transmissible to Transgenic Mice Expressing Homologous Prion Protein.
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- PLoS Pathogens, 2013, v. 9, n. 9, p. 1, doi. 10.1371/journal.ppat.1003643
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Human Prion Disorders: Review of the Current Literature and a Twenty-Year Experience of the National Surveillance Center in the Czech Republic.
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- Diagnostics (2075-4418), 2021, v. 11, n. 10, p. 1821, doi. 10.3390/diagnostics11101821
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The Prion Diseases.
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- Journal of Geriatric Psychiatry & Neurology, 2010, v. 23, n. 4, p. 277, doi. 10.1177/0891988710383576
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PrP knock-out and PrP transgenic mice in prion research.
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- British Medical Bulletin, 2003, v. 66, n. 1, p. 43, doi. 10.1093/bmb/66.1.43
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Case Report: Histopathology and Prion Protein Molecular Properties in Inherited Prion Disease With a De Novo Seven-Octapeptide Repeat Insertion.
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- Frontiers in Cellular Neuroscience, 2020, v. 14, p. 1, doi. 10.3389/fncel.2020.00150
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Gerstmann- Sträussler- Scheinker Syndrome with Variable Phenotype in a New Kindred with PRNP- P102L Mutation.
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- Brain Pathology, 2014, v. 24, n. 2, p. 142, doi. 10.1111/bpa.12083
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Lidské prionové nemoci v ČR: 22 let zkušeností Národní referenční laboratoře pro diagnostiku lidských prionových chorob.
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- Neurologie Pro Praxi, 2024, v. 25, n. 1, p. 9, doi. 10.36290/neu.2024.002
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Prions mediated neurodegenerative disorders.
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- European Review for Medical & Pharmacological Sciences, 2015, v. 19, n. 21, p. 4028
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Clinical Variability in P102L Gerstmann-Sträussler-Scheinker Syndrome.
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- 2019
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- journal article
Case report: A Chinese patient with spinocerebellar ataxia finally confirmed as Gerstmann-Sträussler-Scheinker syndrome with P102L mutation.
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- Frontiers in Neurology, 2023, p. 01, doi. 10.3389/fneur.2023.1187813
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Hyperphosphorylated tau deposition parallels prion protein burden in a case of Gerstmann-Sträussler-Scheinker syndrome P102L mutation complicated with dementia.
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- Acta Neuropathologica, 2002, v. 104, n. 4, p. 342, doi. 10.1007/s00401-002-0547-3
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Extracellular Prion Protein Aggregates in Nine Gerstmann–Sträussler–Scheinker Syndrome Subjects with Mutation P102L: A Micromorphological Study and Comparison with Literature Data.
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- International Journal of Molecular Sciences, 2021, v. 22, n. 24, p. 13303, doi. 10.3390/ijms222413303
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How an Infection of Sheep Revealed Prion Mechanisms in Alzheimer's Disease and Other Neurodegenerative Disorders.
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- International Journal of Molecular Sciences, 2021, v. 22, n. 9, p. 4861, doi. 10.3390/ijms22094861
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Mutations in Prion Protein Gene: Pathogenic Mechanisms in C-Terminal vs. N-Terminal Domain, a Review.
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- International Journal of Molecular Sciences, 2019, v. 20, n. 14, p. 3606, doi. 10.3390/ijms20143606
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Microglia is a component of the prion protein amyloid plaque in the Gerstmann-Sträussler-Scheinker syndrome.
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- Acta Neuropathologica, 1993, v. 85, n. 6, p. 623, doi. 10.1007/BF00334672
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A New Transgenic Mouse Model of Gerstmann-Sträussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP.
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- Journal of Neuroscience, 2009, v. 29, n. 32, p. 10072, doi. 10.1523/JNEUROSCI.2542-09.2009
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Accelerated Accumulation of Misfolded Prion Protein and Spongiform Degeneration in a Drosophila Model of Gerstmann--Sträussler--Scheinker Syndrome.
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- Journal of Neuroscience, 2006, v. 26, n. 48, p. 12408, doi. 10.1523/JNEUROSCI.3372-06.2006
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NMR Structure of the Human Prion Protein with the Pathological Q212P Mutation Reveals Unique Structural Features.
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- PLoS ONE, 2010, v. 5, n. 7, p. 1, doi. 10.1371/journal.pone.0011715
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Loss of Anti-Bax Function in Gerstmann-Strä ussler- Scheinker Syndrome-Associated Prion Protein Mutants.
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- PLoS ONE, 2009, v. 4, n. 8, p. 1, doi. 10.1371/journal.pone.0006647
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Partitioning of human and sheep forms of the pathogenic prion protein during the purification of therapeutic proteins from human plasma.
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- 2002
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- journal article