Results 21 to 30 of about 5,185,837 (206)
Prion-like propagation as a pathogenic principle in frontotemporal dementia. [PDF]
J Neurochem, 2016 AbstractFrontotemporal dementia is a devastating neurodegenerative disease causing stark alterations in personality and language. Characterized by severe atrophy of the frontal and temporal brain lobes, frontotemporal dementia (FTD) shows extreme heterogeneity in clinical presentation, genetic causes, and pathological findings.
Hock EM, Polymenidou M.
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Prion-like propagation of pathology in Parkinson disease. [PDF]
Handb Clin Neurol, 2018 Over 100 years ago, Lewy bodies and Lewy neurites were defined as a pathologic hallmark of Parkinson disease. Eighty years later, α-synuclein was found to be the primary component of these inclusions. Emerging evidence suggests that α-synuclein pathology propagates across interconnected networks throughout the nervous system in a prion-like manner ...
Volpicelli-Daley L, Brundin P.
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Prion-Like Propagation Mechanisms in Tauopathies and Traumatic Brain Injury: Challenges and Prospects. [PDF]
Biomolecules, 2020 The accumulation of tau protein in the form of filamentous aggregates is a hallmark of many neurodegenerative diseases such as Alzheimer’s disease (AD) and chronic traumatic encephalopathy (CTE). These dementias share traumatic brain injury (TBI) as a prominent risk factor. Tau aggregates can transfer between cells and tissues in a “prion-like” manner,
Alyenbaawi H, Allison WT, Mok SA.
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Human-to-mouse prion-like propagation of mutant huntingtin protein. [PDF]
Acta Neuropathol, 2016 Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder of the central nervous system (CNS) that is defined by a CAG expansion in exon 1 of the huntingtin gene leading to the production of mutant huntingtin (mHtt). To date, the disease pathophysiology has been thought to be primarily driven by cell-autonomous mechanisms, but, here,
Jeon I +17 more
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From the prion-like propagation hypothesis to therapeutic strategies of anti-tau immunotherapy. [PDF]
Acta Neuropathol, 2020 Abstract The term “propagon” is used to define proteins that may transmit misfolding in vitro, in tissues or in organisms. Among propagons, misfolded tau is thought to be involved in the pathogenic mechanisms of various “tauopathies” that include Alzheimer's disease, progressive supranuclear palsy, and argyrophilic grain disease. Here, we
Colin M +6 more
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Prion-like propagation of β-amyloid aggregates in the absence of APP overexpression. [PDF]
Acta Neuropathol Commun, 2018 The amyloid cascade hypothesis posits that the initiating event in Alzheimer's disease (AD) is the aggregation and deposition of the β-amyloid (Aβ) peptide, which is a proteolytic cleavage product of the amyloid precursor protein (APP). Mounting evidence suggests that the formation and spread of prion-like Aβ aggregates during AD may contribute to ...
Ruiz-Riquelme A +6 more
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The olfactory bulb as the entry site for prion-like propagation in neurodegenerative diseases. [PDF]
Neurobiol Dis, 2018 Olfactory deficits are present in numerous neurodegenerative disorders and are accompanied by pathology in related brain regions. In several of these disorders, olfactory disturbances appear early and are considered as prodromal symptoms of the disease.
Rey NL, Wesson DW, Brundin P.
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[PSI+] maintenance is dependent on the composition, not primary sequence, of the oligopeptide repeat domain. [PDF]
PLoS ONE, 2011 [PSI(+)], the prion form of the yeast Sup35 protein, results from the structural conversion of Sup35 from a soluble form into an infectious amyloid form.
James A Toombs +4 more
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Scientific Reports, 2022 There is an urgent need to develop disease-modifying therapies to treat neurodegenerative diseases which pose increasing challenges to global healthcare systems.
Madeleine Reilly +7 more
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Human cerebral organoids as a therapeutic drug screening model for Creutzfeldt–Jakob disease
Scientific Reports, 2021 Creutzfeldt–Jakob Disease (CJD) is a fatal, currently incurable, neurodegenerative disease. The search for candidate treatments would be greatly facilitated by the availability of human cell-based models of prion disease. Recently, an induced pluripotent
Bradley R. Groveman +8 more
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