Works matching Gerstmann-Straussler-Scheinker disease
Results: 192
An autopsy report of three kindred in a Gerstmann–Sträussler–Scheinker disease P105L family with a special reference to prion protein, tau, and beta‐amyloid.
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- Brain & Behavior, 2018, v. 8, n. 10, p. N.PAG, doi. 10.1002/brb3.1117
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- Article
Detection of tau in Gerstmann-Sträussler-Scheinker disease (PRNP F198S) by [<sup>18</sup>F]Flortaucipir PET.
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- Acta Neuropathologica Communications, 2018, v. 6, n. 1, p. N.PAG, doi. 10.1186/s40478-018-0608-z
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- 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.
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- PLoS Pathogens, 2018, v. 14, n. 1, p. 1, doi. 10.1371/journal.ppat.1006826
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- Article
Rapamycin Delays Disease Onset and Prevents PrP Plaque Deposition in a Mouse Model of Gerstmann-Sträussler-Scheinker Disease.
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- Journal of Neuroscience, 2012, v. 32, n. 36, p. 12396, doi. 10.1523/JNEUROSCI.6189-11.2012
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- Article
Gerstmann-Straussler-Scheinker disease with PRNP P102L heterozygous mutation presenting as progressive myoclonus epilepsy.
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- European Journal of Neurology, 2017, v. 24, n. 12, p. e87, doi. 10.1111/ene.13447
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- Article
De novo P102L mutation in a patient with Gerstmann-Sträussler-Scheinker disease.
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- European Journal of Neurology, 2011, v. 18, n. 12, p. e152, doi. 10.1111/j.1468-1331.2011.03531.x
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- Article
Gerstmann-Sträussler-Scheinker Disease Presenting with Atypical Parkinsonism, but Typical Magnetic Resonance Imaging Findings of Prion Disease.
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- Movement Disorders Clinical Practice, 2016, v. 3, n. 1, p. 93, doi. 10.1002/mdc3.12228
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- Article
Small Ruminant Nor98 Prions Share Biochemical Features with Human Gerstmann-Sträussler-Scheinker Disease and Variably Protease-Sensitive Prionopathy.
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- PLoS ONE, 2013, v. 8, n. 6, p. 1, doi. 10.1371/journal.pone.0066405
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- Article
Rare health conditions 26: fatal familial insomnia, Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker syndrome.
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- British Journal of Healthcare Assistants, 2019, v. 13, n. 8, p. 370, doi. 10.12968/bjha.2019.13.8.370
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- Article
БЪЛГАРСКА ФАМИЛИЯ С БОЛЕСТ НА GERSTMANN-STRAUSSLER-SCHEINKER И Р.P102L МУТАЦИЯ В PRNP ГЕНА
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- Medical Review / Meditsinski Pregled, 2024, v. 60, n. 2, p. 55
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- Article
Isolation of infectious, non-fibrillar and oligomeric prions from a genetic prion disease.
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- 2020
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- Publication type:
- journal article
Report on the first Chinese family with Gerstmann-Sträussler-Scheinker disease manifesting the codon 102 mutation in the prion protein gene.
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- Neuropathology, 2006, v. 26, n. 5, p. 429, doi. 10.1111/j.1440-1789.2006.00704.x
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- Article
Ultrastructural study of florid plaques in variant Creutzfeldt–Jakob disease: a comparison with amyloid plaques in kuru, sporadic Creutzfeldt–Jakob disease and Gerstmann–Sträussler–Scheinker disease.
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- Neuropathology & Applied Neurobiology, 2009, v. 35, n. 1, p. 46, doi. 10.1111/j.1365-2990.2008.00959.x
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- Article
Dopaminergic neurodegeneration in Gerstmann–Sträussler–Scheinker (P102L) disease: insights from imaging and pathological examination.
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- Frontiers in Neurology, 2024, p. 1, doi. 10.3389/fneur.2024.1452709
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- Article
Gait apraxia as a presenting sign of Gerstmann‐Sträussler‐Scheinker disease.
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- Neurology & Clinical Neuroscience, 2021, v. 9, n. 4, p. 339, doi. 10.1111/ncn3.12511
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- Article
Gerstmann‐Sträussler‐Scheinker Disease Presenting as Late‐Onset Slowly Progressive Spinocerebellar Ataxia, and Comparative Case Series with Neuropathology.
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- Movement Disorders Clinical Practice, 2024, v. 11, n. 4, p. 411, doi. 10.1002/mdc3.13976
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- Article
Description of the first Spanish case of Gerstmann–Sträussler–Scheinker disease with A117V variant: clinical, histopathological and biochemical characterization.
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- Journal of Neurology, 2022, v. 269, n. 8, p. 4253, doi. 10.1007/s00415-022-11051-9
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- Article
Gerstmann-Sträussler-Scheinker disease showing β-protein type cerebellar and cerebral amyloid angiopathy.
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- Acta Neuropathologica, 1994, v. 88, n. 3, p. 262, doi. 10.1007/BF00293403
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- Article
Dopaminergic neurodegeneration in Gerstmann--Sträussler--Scheinker (P102L) disease: insights from imaging and pathological examination.
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- Frontiers in Neurology, 2024, p. 01, doi. 10.3389/fneur.2024.1452709
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- Article
Juvenile Gerstmann‐Sträussler‐Scheinker Disease Mimicking Anticipation Phenomenon.
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- Movement Disorders Clinical Practice, 2023, v. 10, n. 11, p. 1700, doi. 10.1002/mdc3.13878
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- Article
A novel seven-octapeptide repeat insertion in the prion protein gene ( PRNP) in a Dutch pedigree with Gerstmann-Sträussler-Scheinker disease phenotype: comparison with similar cases from the literature.
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- Acta Neuropathologica, 2011, v. 121, n. 1, p. 59, doi. 10.1007/s00401-010-0656-3
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- Article
Gerstmann-Sträussler-Scheinker disease with the Q217R mutation mimicking frontotemporal dementia.
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- Acta Neuropathologica, 2005, v. 110, n. 3, p. 317, doi. 10.1007/s00401-005-1054-0
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- Article
A case of sporadic Creutzfeldt-Jakob disease with a Gerstmann-Sträussler-Scheinker phenotype but no alterations in the PRNP gene.
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- Acta Neuropathologica, 1998, v. 96, n. 4, p. 425, doi. 10.1007/s004010050915
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- Article
Cerebellar plaques in familial Alzheimer's disease (Gerstmann-Sträussler-Scheinker variant?).
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- Acta Neuropathologica, 1985, v. 65, n. 3/4, p. 235, doi. 10.1007/BF00687003
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- Article
Unusual Clinical and Molecular-Pathological Profile of Gerstmann-Sträussler-Scheinker Disease Associated With a Novel PRNP Mutation (V176G).
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- JAMA Neurology, 2013, v. 70, n. 9, p. 1180, doi. 10.1001/jamaneurol.2013.165
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- Article
Gerstmann-Sträussler-Scheinker disease: Immunohistological and experimental studies.
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- Annals of Neurology, 1988, v. 24, n. 1, p. 35, doi. 10.1002/ana.410240108
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- Article
Gerstmann–Sträussler–Scheinker Disease with F198S Mutation Induces Independent Tau and Prion Protein Pathologies in Bank Voles.
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- Biomolecules (2218-273X), 2022, v. 12, n. 10, p. N.PAG, doi. 10.3390/biom12101537
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- Article
Brain microglia were activated in sporadic CJD but almost unchanged in fatal familial insomnia and G114V genetic CJD.
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- Virology Journal, 2013, v. 10, n. 1, p. 1, doi. 10.1186/1743-422X-10-216
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- Article
Gerstmann-Sträussler-Scheinker Disease: A Case Report.
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- Journal of the Korean Society of Radiology (2951-0805), 2023, v. 84, n. 3, p. 745, doi. 10.3348/jksr.2022.0089
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- Article
Localization of A11-reactive oligomeric species in prion diseases.
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- Histopathology, 2013, v. 62, n. 7, p. 994, doi. 10.1111/his.12097
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- Article
Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS.
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- PLoS Pathogens, 2015, v. 11, n. 7, p. 1, doi. 10.1371/journal.ppat.1004953
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- Article
PET of Brain Prion Protein Amyloid in Gerstmann–Sträussler–Scheinker Disease.
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- Brain Pathology, 2010, v. 20, n. 2, p. 419, doi. 10.1111/j.1750-3639.2009.00306.x
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- Article
Correction to "Gerstmann‐Sträussler‐Scheinker Disease Presenting as Late‐Onset Slowly Progressive Spinocerebellar Ataxia, and Comparative Case Series with Neuropathology".
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- Movement Disorders Clinical Practice, 2024, v. 11, n. 9, p. 1177, doi. 10.1002/mdc3.14058
- Publication type:
- Article
Gerstmann-Sträussler-Scheinker disease: Autopsy study of a familial case.
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- Annals of Neurology, 1986, v. 20, n. 4, p. 540, doi. 10.1002/ana.410200418
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- Article
Gerstmann-Sträussler-Scheinker disease with coincidental familial onset.
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- Annals of Neurology, 1983, v. 14, n. 6, p. 670, doi. 10.1002/ana.410140611
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- Article
Cryo-EM structures of prion protein filaments from Gerstmann–Sträussler–Scheinker disease.
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- Acta Neuropathologica, 2022, v. 144, n. 3, p. 509, doi. 10.1007/s00401-022-02461-0
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- Article
Neuropathological features of a case with schizophrenia and prion protein gene P102L mutation before onset of Gerstmann-Sträussler-Scheinker disease.
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- Acta Neuropathologica, 2003, v. 106, n. 1, p. 92, doi. 10.1007/s00401-003-0697-y
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- Article
Re: Variable Phenotype in Gerstmann-Sträussler-Scheinker Disease.
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- 2004
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- Letter
Upregulation of Micro RNA-146a (miRNA-146a), A Marker for Inflammatory Neurodegeneration, in Sporadic Creutzfeldt–Jakob Disease (sCJD) and Gerstmann–Straussler–Scheinker (GSS) Syndrome.
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- Journal of Toxicology & Environmental Health: Part A, 2011, v. 74, n. 22-24, p. 1460, doi. 10.1080/15287394.2011.618973
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- Article
Ilya Mark Scheinker: Controversial Neuroscientist and Refugee From National Socialist Europe.
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- Canadian Journal of Neurological Sciences, 2016, v. 43, n. 2, p. 334, doi. 10.1017/cjn.2015.359
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- Article
A case of Gerstmann-Sträussler-Scheinker disease with a novel six octapeptide repeat insertion.
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- 2011
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- Other
Atypical frontotemporal dementia as a new clinical phenotype of Gerstmann-Straussler-Scheinker disease with the PrP-P102L mutation. Description of a previously unreported Italian family.
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- Neurological Sciences, 2008, v. 29, n. 6, p. 405, doi. 10.1007/s10072-008-1025-z
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- Article
Prions in dentistry: A need to be concerned and known.
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- Journal of Oral & Maxillofacial Pathology (0973029X), 2016, v. 20, n. 1, p. 111, doi. 10.4103/0973-029X.180961
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- Article
Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments.
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- Acta Neuropathologica Communications, 2019, v. 7, n. 1, p. N.PAG, doi. 10.1186/s40478-019-0734-2
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- Article
Neurotoxic and Gliotrophic Activity of a Synthetic Peptide Homologous to Gerstmann-Sträussler-Scheinker Disease Amyloid Protein.
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- Journal of Neuroscience, 2007, v. 27, n. 7, p. 1576, doi. 10.1523/JNEUROSCI.5145-06.2007
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- Article
“With a Smile Through Tears”: The Uprooted Career of the Man Behind Gerstmann Syndrome.
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- Journal of the History of the Neurosciences, 2015, v. 24, n. 2, p. 148, doi. 10.1080/0964704X.2014.950089
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- Article
Allelic Origin of Protease-Sensitive and Protease-Resistant Prion Protein Isoforms in Gerstmann-Sträussler-Scheinker Disease with the P102L Mutation.
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- PLoS ONE, 2012, v. 7, n. 2, p. 1, doi. 10.1371/journal.pone.0032382
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- Article
Unusual presentations and intrafamilial phenotypic variability in infantile onset Alexander disease.
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- 2016
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- Publication type:
- journal article
Divergent clinical and neuropathological phenotype in a Gerstmann- Sträussler- Scheinker P102 L family.
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- Acta Neurologica Scandinavica, 2012, v. 126, n. 5, p. 315, doi. 10.1111/j.1600-0404.2011.01628.x
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- Article
A previously undiagnosed case of Gerstmann-Sträussler-Scheinker disease revealed by PRNP gene analysis in patients with adult-onset ataxia.
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- 2008
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- Publication type:
- journal article