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Genetic prion diseases presenting as frontotemporal dementia: clinical features and diagnostic challenge
BackgroundTo elucidate the clinical and ancillary features of genetic prion diseases (gPrDs) presenting with frontotemporal dementia (FTD) to aid...
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The E3 Ubiquitin Ligase TRAF6 Interacts with the Cellular Prion Protein and Modulates Its Solubility and Recruitment to Cytoplasmic p62/SQSTM1-Positive Aggresome-Like Structures
The cellular prion protein (PrP C ) is a ubiquitous glycoprotein highly expressed in the brain where it is involved in neurite outgrowth, copper...
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Role of sialylation of N-linked glycans in prion pathogenesis
Mammalian prion or PrP Sc is a proteinaceous infectious agent that consists of a misfolded, self-replicating state of the prion protein or PrP C . PrP C ...
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Slow Virus and Prion Diseases
Slow virus diseases are a large group of related neurodegenerative conditions which affect both humans and animals. The diseases are caused by a...
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Decrease in Skin Prion-Seeding Activity of Prion-Infected Mice Treated with a Compound Against Human and Animal Prions: a First Possible Biomarker for Prion Therapeutics
Previous studies have revealed that the infectious scrapie isoform of prion protein (PrP Sc ) harbored in the skin tissue of patients or animals with...
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Prion-induced photoreceptor degeneration begins with misfolded prion protein accumulation in cones at two distinct sites: cilia and ribbon synapses
Accumulation of misfolded host proteins is central to neuropathogenesis of numerous human brain diseases including prion and prion-like diseases....
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Sporadic Creutzfeldt-Jakob disease VM1: phenotypic and molecular characterization of a novel subtype of human prion disease
The methionine (M)—valine (V) polymorphic codon 129 of the prion protein gene ( PRNP ) plays a central role in both susceptibility and phenotypic...
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Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia
In sporadic Creutzfeldt–Jakob disease (sCJD), the pathological changes appear to be restricted to the central nervous system. Only involvement of the...
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Extracellular vesicles with diagnostic and therapeutic potential for prion diseases
Prion diseases (PrD) or transmissible spongiform encephalopathies (TSE) are invariably fatal and pathogenic neurodegenerative disorders caused by the...
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Gene expression and epigenetic markers of prion diseases
Epigenetics, meaning the variety of mechanisms underpinning gene regulation and chromatin states, plays a key role in normal development as well as...
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Pharmacological modulation of TSPO in microglia/macrophages and neurons in a chronic neurodegenerative model of prion disease
Neuroinflammation is an important component of many neurodegenerative diseases, whether as a primary cause or a secondary outcome. For that reason,...
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Cell biology of prion strains in vivo and in vitro
The properties of infectious prions and the pathology of the diseases they cause are dependent upon the unique conformation of each prion strain. How...
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Anti-prion Drugs Targeting the Protein Folding Activity of the Ribosome Reduce PABPN1 Aggregation
Prion diseases are caused by the propagation of PrP Sc , the pathological conformation of the PrP C prion protein. The molecular mechanisms underlying...
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Organoids for modeling prion diseases
Human cerebral organoids are an exciting and novel model system emerging in the field of neurobiology. Cerebral organoids are spheres of...
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Volatile Anesthetic Sevoflurane Precursor 1,1,1,3,3,3-Hexafluoro-2-Propanol (HFIP) Exerts an Anti-Prion Activity in Prion-Infected Culture Cells
Prion disease is a neurodegenerative disorder with progressive neurologic symptoms and accelerated cognitive decline. The causative protein of prion...
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Cellular Prion Protein Attenuates OGD/R-Induced Damage by Skewing Microglia toward an Anti-inflammatory State via Enhanced and Prolonged Activation of Autophagy
Modulation of microglial pro/anti-inflammatory states and autophagy are promising new therapies for ischemic stroke, but the underlying mechanisms...
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Mutation of copper binding sites on cellular prion protein abolishes its inhibitory action on NMDA receptors in mouse hippocampal neurons
We have previously reported that cellular prion protein (PrP C ) can down-regulate NMDA receptor activity and in a copper dependent manner. Here, we...
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A multiverse of α-synuclein: investigation of prion strain properties with carboxyl-terminal truncation specific antibodies in animal models
Synucleinopathies are a group of neurodegenerative disorders characterized by the presence of misfolded α-Synuclein (αSyn) in the brain. These...
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RAB7A GTPase Is Involved in Mitophagosome Formation and Autophagosome–Lysosome Fusion in N2a Cells Treated with the Prion Protein Fragment 106–126
Failed communication between mitochondria and lysosomes causes dysfunctional mitochondria, which may induce mitochondria-related neurodegenerative...
