Cargando…

Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans

Prion diseases are a unique group of rare neurodegenerative disorders characterized by tissue deposition of heterogeneous aggregates of abnormally folded protease-resistant prion protein (PrP(Sc)), a broad spectrum of disease phenotypes and a variable efficiency of disease propagation in vivo. The d...

Descripción completa

Detalles Bibliográficos
Autores principales:	Rossi, Marcello, Baiardi, Simone, Parchi, Piero
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2019
Materias:	Review
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6520670/ https://www.ncbi.nlm.nih.gov/pubmed/30934971 http://dx.doi.org/10.3390/v11040309

Ejemplares similares

The Use of Real-Time Quaking-Induced Conversion for the Diagnosis of Human Prion Diseases
por: Poleggi, Anna, et al.
Publicado: (2022)

Detection of prions in skin punch biopsies of Creutzfeldt–Jakob disease patients
por: Mammana, Angela, et al.
Publicado: (2020)

Prion Protein Misfolding, Strains, and Neurotoxicity: An Update from Studies on Mammalian Prions
por: Poggiolini, Ilaria, et al.
Publicado: (2013)

Towards an improved early diagnosis of neurodegenerative diseases: the emerging role of in vitro conversion assays for protein amyloids
por: Candelise, Niccolò, et al.
Publicado: (2020)

Revisiting the Heidenhain Variant of Creutzfeldt-Jakob Disease: Evidence for Prion Type Variability Influencing Clinical Course and Laboratory Findings
por: Baiardi, Simone, et al.
Publicado: (2015)

The characterization of AD/PART co-pathology in CJD suggests independent pathogenic mechanisms and no cross-seeding between misfolded Aβ and prion proteins
por: Rossi, Marcello, et al.
Publicado: (2019)

CSF Ubiquitin Levels Are Higher in Alzheimer’s Disease than in Frontotemporal Dementia and Reflect the Molecular Subtype in Prion Disease
por: Abu-Rumeileh, Samir, et al.
Publicado: (2020)

Diagnostic and prognostic performance of CSF α‐synuclein in prion disease in the context of rapidly progressive dementia
por: Mastrangelo, Andrea, et al.
Publicado: (2021)

High diagnostic value of second generation CSF RT-QuIC across the wide spectrum of CJD prions
por: Franceschini, Alessia, et al.
Publicado: (2017)

RT‐QuIC Detection of Pathological α‐Synuclein in Skin Punches of Patients with Lewy Body Disease
por: Mammana, Angela, et al.
Publicado: (2021)

Atypical Creutzfeldt-Jakob disease with PrP-amyloid plaques in white matter: molecular characterization and transmission to bank voles show the M1 strain signature
por: Rossi, Marcello, et al.
Publicado: (2017)

Prion-specific and surrogate CSF biomarkers in Creutzfeldt-Jakob disease: diagnostic accuracy in relation to molecular subtypes and analysis of neuropathological correlates of p-tau and Aβ42 levels
por: Lattanzio, Francesca, et al.
Publicado: (2017)

Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein
por: Race, Brent, et al.
Publicado: (2018)

Antemortem CSF Aβ42/Aβ40 ratio predicts Alzheimer's disease pathology better than Aβ42 in rapidly progressive dementias
por: Baiardi, Simone, et al.
Publicado: (2018)

Phenotypic Heterogeneity of Variably Protease-Sensitive Prionopathy: A Report of Three Cases Carrying Different Genotypes at PRNP Codon 129
por: Baiardi, Simone, et al.
Publicado: (2022)

Two novel PRNP truncating mutations broaden the spectrum of prion amyloidosis
por: Capellari, Sabina, et al.
Publicado: (2018)

Sporadic Creutzfeldt-Jakob disease VM1: phenotypic and molecular characterization of a novel subtype of human prion disease
por: Gelpi, Ellen, et al.
Publicado: (2022)

Defining the phenotypic spectrum of sporadic Creutzfeldt–Jakob disease MV2K: the kuru plaque type
por: Baiardi, Simone, et al.
Publicado: (2023)

Genomic, transcriptomic and RNA editing analysis of human MM1 and VV2 sporadic Creutzfeldt-Jakob disease
por: Tarozzi, Martina, et al.
Publicado: (2022)

Strain-Dependent Morphology of Reactive Astrocytes in Human- and Animal-Vole-Adapted Prions
por: Bruno, Rosalia, et al.
Publicado: (2023)

Two distinct prions in fatal familial insomnia and its sporadic form
por: Takeuchi, Atsuko, et al.
Publicado: (2019)

Cerebrospinal Fluid and Blood Neurofilament Light Chain Protein in Prion Disease and Other Rapidly Progressive Dementias: Current State of the Art
por: Abu-Rumeileh, Samir, et al.
Publicado: (2021)

Clinicopathological features of the rare form of Creutzfeldt-Jakob disease in R208H-V129V PRNP carrier
por: Tiple, Dorina, et al.
Publicado: (2019)

146 Understanding the pathogenesis of prion and surveillance for human prion diseases in China
por: Dong, Xiao-Ping
Publicado: (2014)

Differential Accumulation of Misfolded Prion Strains in Natural Hosts of Prion Diseases
por: Lambert, Zoe J., et al.
Publicado: (2021)

Review: Contribution of transgenic models to understanding human prion disease
por: Wadsworth, J D F, et al.
Publicado: (2010)

CSF biomarkers of neuroinflammation in distinct forms and subtypes of neurodegenerative dementia
por: Abu-Rumeileh, Samir, et al.
Publicado: (2019)

Gerstmann-Sträussler-Scheinker disease (PRNP p.D202N) presenting with atypical parkinsonism
por: Baiardi, Simone, et al.
Publicado: (2020)

Structural evidence for the critical role of the prion protein hydrophobic region in forming an infectious prion
por: Abskharon, Romany, et al.
Publicado: (2019)

Diagnostic and prognostic value of cerebrospinal fluid SNAP-25 and neurogranin in Creutzfeldt-Jakob disease in a clinical setting cohort of rapidly progressive dementias
por: Bentivenga, Giuseppe Mario, et al.
Publicado: (2023)

Evidence for preexisting prion substrain diversity in a biologically cloned prion strain
por: Gunnels, Tess, et al.
Publicado: (2023)

Toward a Better Understanding of Human Prion Diseases
Publicado: (2006)

Evidence for Varied Aetiologies Regulating the Transmission of Prion Disease: Implications for Understanding the Heritable Basis of Prion Incubation Times
por: Iyegbe, Conrad O., et al.
Publicado: (2010)

Clinical effects of Lewy body pathology in cognitively impaired individuals
por: Quadalti, Corinne, et al.
Publicado: (2023)

Glycoform-Selective Prion Formation in Sporadic and Familial Forms of Prion Disease
por: Xiao, Xiangzhu, et al.
Publicado: (2013)

Prion Protein: The Molecule of Many Forms and Faces
por: Kovač, Valerija, et al.
Publicado: (2022)

An overview of human prion diseases
por: Imran, Muhammad, et al.
Publicado: (2011)

Prion and Prion-Like Protein Strains: Deciphering the Molecular Basis of Heterogeneity in Neurodegeneration
por: Scialò, Carlo, et al.
Publicado: (2019)

Correction: Glycoform-Selective Prion Formation in Sporadic and Familial Forms of Prion Disease
por: Xiao, Xiangzhu, et al.
Publicado: (2013)

Prion Strains and Transmission Barrier Phenomena
por: Igel-Egalon, Angélique, et al.
Publicado: (2018)

Cannot write session to /tmp/vufind_sessions/sess_hltukc789uakv7l3qhirv3jbpt