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Potential Therapeutic Use of Stem Cells for Prion Diseases
Prion diseases are neurodegenerative disorders that are progressive, incurable, and deadly. The prion consists of PrPSc, the misfolded pathogenic isoform of the cellular prion protein (PrPC).
Mohammed Zayed+2 more
doaj +1 more source
Cellular Prion Protein Mediates Toxic Signaling of Amyloid Beta [PDF]
Prion diseases in humans and animals comprise a group of invariably fatal neurodegenerative diseases characterized by the formation of a pathogenic protein conformer designated PrPSc and infectious particles denoted prions.
Resenberger, Ulrike K.+2 more
core +1 more source
Hepatic endothelial Alk1 signaling protects from development of vascular malformations while maintaining organ‐specific endothelial differentiation and angiocrine portmanteau of the names Wingless and Int‐1 signaling. Abstract Background and Aims In hereditary hemorrhagic telangiectasia (HHT), severe liver vascular malformations are associated with ...
Christian David Schmid+20 more
wiley +1 more source
Differential Accumulation of Misfolded Prion Strains in Natural Hosts of Prion Diseases
Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of neurodegenerative protein misfolding diseases that invariably cause death.
Zoe J. Lambert+3 more
doaj +1 more source
Alzheimer's disease-related phenotypes in mice can be rescued by blockade of either cellular prion protein or metabotropic glutamate receptor 5. We sought genetic and biochemical evidence that these proteins function cooperatively as an obligate complex ...
L. Haas+5 more
semanticscholar +1 more source
A Sequence-Dependent DNA Condensation Induced by Prion Protein
Different studies indicated that the prion protein induces hybridization of complementary DNA strands. Cell culture studies showed that the scrapie isoform of prion protein remained bound with the chromosome. In present work, we used an oxazole dye, YOYO,
Alakesh Bera, Sajal Biring
doaj +1 more source
A quantitative characterization of interaction between prion protein with nucleic acids
Binding of recombinant prion protein with small highly structured RNAs, prokaryotic and eukaryotic prion protein mRNA pseudoknots, tRNA and polyA has been studied by the change in fluorescence anisotropy of the intrinsic tryptophan groups of the protein.
Alakesh Bera, Sajal Biring
doaj +1 more source
Intraperitoneal Infection of Wild-Type Mice with Synthetically Generated Mammalian Prion. [PDF]
The prion hypothesis postulates that the infectious agent in transmissible spongiform encephalopathies (TSEs) is an unorthodox protein conformation based agent.
Xinhe Wang+6 more
doaj +1 more source
Distinct amino acid compositional requirements for formation and maintenance of the [PSI+] prion in yeast [PDF]
Multiple yeast prions have been identified that result from the structural conversion of proteins into a self-propagating amyloid form. Amyloid-based prion activity in yeast requires a series of discrete steps.
Ben-Musa, Zobaida+6 more
core +2 more sources
Prion-Like Propagation of Protein Misfolding and Aggregation in Amyotrophic Lateral Sclerosis
The discovery that prion protein can misfold into a pathological conformation that encodes structural information capable of both propagation and inducing severe neuropathology has revolutionized our understanding of neurodegenerative disease.
Luke McAlary+3 more
semanticscholar +1 more source