Prion-Like Propagation of Protein Misfolding and Aggregation in Amyotrophic Lateral Sclerosis [PDF]
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 +6 more
doaj +5 more sources
What is the evidence that tau pathology spreads through prion-like propagation? [PDF]
Emerging experimental evidence suggests that the spread of tau pathology in the brain in Tauopathies reflects the propagation of abnormal tau species along neuroanatomically connected brain areas.
Amrit Mudher +10 more
doaj +11 more sources
From Prion Diseases to Prion-Like Propagation Mechanisms of Neurodegenerative Diseases [PDF]
Prion diseases are fatal neurodegenerative sporadic, inherited, or acquired disorders. In humans, Creutzfeldt-Jakob disease is the most studied prion disease.
Isabelle Acquatella-Tran Van Ba +2 more
doaj +6 more sources
TAF15 amyloids propagate via defined motifs in a prion-like fashion [PDF]
TATA-box binding protein–associated factor 15 (TAF15) is an RNA-binding protein and the primary fibrillar constituent in a subset of frontotemporal lobar degeneration (FTLD) cases.
Katerina Konstantoulea +16 more
doaj +3 more sources
The Effect of Fragmented Pathogenic α-Synuclein Seeds on Prion-like Propagation. [PDF]
Aggregates of abnormal proteins are widely observed in neuronal and glial cells of patients with various neurodegenerative diseases, and it has been proposed that prion-like behavior of these proteins can account for not only the onset but also the progression of these diseases.
Tarutani A +6 more
europepmc +5 more sources
Prion-like propagation of protein aggregation and related therapeutic strategies. [PDF]
Many neurodegenerative diseases are characterized by the progressive accumulation of aggregated protein. Recent evidence suggests the prion-like propagation of protein misfolding underlies the spread of pathology observed in these diseases. This review traces our understanding of the mechanisms that underlie this phenomenon and discusses related ...
Kaufman SK, Diamond MI.
europepmc +5 more sources
Dementia is one of the leading causes of death worldwide, with tauopathies, a class of diseases defined by pathology associated with the microtubule-enriched protein, tau, as the major contributor.
Zein Amro +2 more
doaj +3 more sources
Prognostic role of ‘prion-like propagation’ in SOD1-linked familial ALS: an alternative view [PDF]
‘Prion-like propagation’ has recently been proposed for disease spread in Cu/Zn superoxide dismutase 1 (SOD1)-linked familial amyotrophic lateral sclerosis (ALS). Pathological SOD1 conformers are presumed to propagate via cell-to-cell transmission.
Keizo eSugaya, Imaharu eNakano
doaj +2 more sources
Increasing evidence suggests that neurodegenerative disorders share a common pathogenic feature: the presence of deposits of misfolded proteins with altered physicochemical properties in the Central Nervous System.
Carlo Scialò +3 more
doaj +3 more sources
The copper transport-associated protein Ctr4 can form prion-like epigenetic determinants in Schizosaccharomyces pombe [PDF]
Prions are protein-based infectious entities associated with fatal brain diseases in animals, but also modify a range of host-cell phenotypes in the budding yeast, Saccharomyces cerevisiae. Many questions remain about the evolution and biology of prions.
Theodora Sideri +6 more
doaj +2 more sources

