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A Corticobasal Syndrome Variant of Familial Creutzfeldt-Jakob Disease with Stroke-Like Onset
Published 2016-01-01“…Creutzfeldt-Jakob disease (CJD) is an untreatable rare human prion disease characterized by rapidly progressive dementia along with various neurological features, including myoclonus and sometimes other movement disorders. …”
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RNA-Binding Proteins in Amyotrophic Lateral Sclerosis and Neurodegeneration
Published 2012-01-01“…While the causes of this devastating disease are poorly understood, recent advances have implicated RNA-binding proteins (RBPs) that contain predicted prion domains as a major culprit. Specifically, mutations in the RBPs TDP-43 and FUS can cause ALS. …”
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Preface
Published 2015-03-01“…Webb.The topics of the 12 articles appearing in this special issue includeevolutionary dynamics of population growth, spatio-temporal dynamicsin reaction-diffusion biological models, transmission dynamics ofinfectious diseases, modeling of antibiotic-resistant bacteria in hospitals,analysis of Prion models, age-structured models in ecology and epidemiology,modeling of immune response to infections, modeling of cancer growth, etc.These topics partially represent the broad areas of Glenn's research interest.For more information please click the “Full Text” above.…”
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Synaptic Paths to Neurodegeneration: The Emerging Role of TDP-43 and FUS in Synaptic Functions
Published 2018-01-01“…Furthermore, the ability of the low-complexity prion-like domains of TDP-43 and FUS to form liquid droplets suggests a potential mechanism for mRNP assembly and conversion. …”
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45
Toll-Like Receptors Expression and Signaling in Glia Cells in Neuro-Amyloidogenic Diseases: Towards Future Therapeutic Application
Published 2010-01-01“…This paper will focus on TLR signaling in glial cells in neurodegenerative diseases such as Alzheimer's disease, prion diseases, amyotrophic lateral sclerosis, and Parkinson's disease. …”
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Down but Not Out: The Consequences of Pretangle Tau in the Locus Coeruleus
Published 2017-01-01“…In this review, we combine the literature on how pathogenic tau affects forebrain neurons with the known properties and degeneration patterns of LC neurons to synthesize hypotheses on hyperphosphorylated tau-induced dysfunction of LC neurons and the prion-like spread of pretangle tau from the LC to the forebrain. …”
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The Contribution of α-Synuclein Spreading to Parkinson’s Disease Synaptopathy
Published 2017-01-01“…Indeed, monomeric, oligomeric, and fibrillary α-synuclein can move from cell to cell and can trigger the aggregation of the endogenous protein in recipient neurons. This novel “prion-like” behavior could further contribute to synaptic failure in PD and other synucleinopathies. …”
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Creutzfeldt-Jakob Disease and Infection Control
Published 2001-01-01“…However, a recent American publication made recommendations on what procedures should be used for reprocessing items that have been in contact with the prion protein (PrP) (4). These recommendations differ substantially from the draft Canadian guidelines. …”
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Recursive seed amplification detects distinct α-synuclein strains in cerebrospinal fluid of patients with Parkinson’s disease
Published 2025-01-01“…Pathologically, it is characterized by neuronal inclusions containing misfolded, fibrillar alpha-synuclein (aSyn). Prion-like properties of aSyn contribute to the spread of aSyn pathology throughout the nervous system as the disease progresses. …”
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50
The doppel (Dpl) Protein Influences In Vitro Migration Capability in Astrocytoma-Derived Cells
Published 2008-01-01“…Doppel (Dpl) protein is the paralogue of the cellular prion (PrPC) protein. In humans, Dpl is expressed almost exclusively in testis where it is involved in spermatogenesis. …”
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51
Bioinformatics Analysis of Exercise-Related Biomarkers in Diabetes
Published 2022-01-01“…Then, the GSEA analysis showed that 6 KEGG pathways were associated with postexercise prediabetic samples, namely, ABC transporters, focal adhesion, MAPK signaling pathway, prion diseases, melanogenesis, and gap junction. …”
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52
Human Islet Amyloid Polypeptide Fibril Binding to Catalase: A Transmission Electron Microscopy and Microplate Study
Published 2010-01-01“…Human IAPP fibrils are toxic in a similar manner to Alzheimer's amyloid-β (Aβ) and prion protein (PrP) fibrils. Previous studies have shown that catalase binds to Aβ fibrils and appears to recognize a region containing the Gly-Ala-Ile-Ile sequence that is similar to the Gly-Ala-Ile-Leu sequence found in human IAPP residues 24-27. …”
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Pengembangan dan Efektivitas Buku Panduan Konseling Karir Ginzberg dengan Teknik Modeling untuk Memilih Studi Lanjut Siswa
Published 2022-12-01“…The results of the study concluded that the results obtained from the validity test using Lawshe, Gilbert, G. E., & Prion, S. (2016) CVR and CVI showed a per-item value greater than 0 (CVR>0). …”
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Brain-derived tau oligomer polymorphs: distinct aggregations, stability profiles, and biological activities
Published 2025-01-01“…Tau oligomers are suggested to be the primary neurotoxic species that initiate aggregation and propagate prion-like structures. Furthermore, different diseases are shown to have distinct structural characteristics of aggregated tau, denoted as polymorphs. …”
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Early Delivery of Misfolded PrP from ER to Lysosomes by Autophagy
Published 2013-01-01“…Prion diseases are linked to the accumulation of a misfolded isoform (PrPSc) of prion protein (PrP). …”
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Pharmacological Modulation of Three Modalities of CA1 Hippocampal Long-Term Potentiation in the Ts65Dn Mouse Model of Down Syndrome
Published 2018-01-01“…Because 4xHFS-induced LTP had not yet been characterized in Ts65Dn mice, we also investigated the effects of picrotoxin, amyloid beta oligomers, and soluble recombinant human prion protein (rPrP) on this form of LTP. Whereas ≥10 μM picrotoxin increased LTP to control levels, it also caused seizure-like oscillations. …”
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Iatrogenic cerebral amyloid angiopathy and Alzheimer's disease co‐pathology
Published 2025-01-01“…Abstract Iatrogenic cerebral amyloid angiopathy, a disease caused by contact with neurosurgical material or human growth hormone contaminated by beta‐amyloid peptide (Aβ), has a prion‐like transmission mechanism. We present a series of three patients under 55 years of age who underwent cranial surgery. …”
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Halloysite clay nanotubes as platforms for loading of aptamers and antisense oligonucleotides
Published 2025-03-01“…In this study, halloysite clay nanotubes have been investigated as a platform for the encapsulation of an antisense oligonucleotide (ASO1) and a DNA aptamer (D12) with potential in the treatment of prion diseases. The loading of both molecules within halloysite cavity has been conducted by using the cyclic vacuum assisted protocol in aqueous medium. …”
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Distinct regulation of Tau Monomer and aggregate uptake and intracellular accumulation in human neurons
Published 2024-12-01“…Abstract Background The prion-like spreading of Tau pathology is the leading cause of disease progression in various tauopathies. …”
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