To date, a total of 13 prion diseases have been recognized in man and animals. The human diseases are: Kuru, Creutzfeldt-Jakob disease (CJD), variant CJD, Gertmann-Straussler-Scheinker Syndrome, fatal familial insomnia and Alpers’ disease. The animal diseases are: scrapie, transmissible mink encephalopathy, chronic wasting disease, bovine spongiform encephalopathy, feline spongiform encephalopa...

Growing evidence indicates that small extracellular vesicles, called exosomes, are prominent mediators of neurodegenerative diseases such as prion, Alzheimer's and Parkinson's disease. Exosomes contain neurodegenerative disease associated proteins such as the prion protein, β-amyloid and α-synuclein. Only demonstrated so far in vivo with prion disease, exosomes are hypothesised to also facilita...

Prion diseases are progressive neurodegenerative disorders affecting humans and various mammals. The prominent neuropathological change in prion-affected brains is neuroinflammation, histopathologically characterized by reactive gliosis surrounding prion deposition. cause effect of these cellular responses still unclear. Here we investigate the impact innate immune on replication using vitro ce...

Prion diseases are invariably fatal and highly infectious neurodegenerative diseases affecting humans and animals. The neurodegenerative diseases such as Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob diseases, Gerstmann-Sträussler-Scheinker syndrome, Fatal Familial Insomnia, Kuru in humans, scrapie in sheep, bovine spongiform encephalopathy (or ‘mad-cow’ disease) and chronic wasting dise...

Prion diseases are neurodegenerative conditions caused by misfolding of the prion protein, leading to conspicuous neuronal loss and intense microgliosis. Recent experimental evidence point towards a protective role of microglia against prion-induced neurodegeneration, possibly through elimination of prion-containing apoptotic bodies. The molecular mechanisms by which microglia recognize and eli...

Rapidly progressive dementias are a rare group of cognitive disorders that primarily require the exclusion large number potentially reversible causes. Prion diseases frequent in this disorders. Some hereditary forms Alzheimer's disease can also be aggressive, with onset at young age and autosomal dominant inheritance family. The article presents first case report patient verified Ile143Thr muta...

Prion diseases are fatal, transmissible neurodegenerative diseases of the central nervous system. An abnormally protease-resistant and insoluble form (PrP(Sc)) of the normally soluble protease-sensitive host prion protein (PrP(C)) is the major component of the infectious prion. During the course of prion disease, PrP(Sc) accumulates primarily in the lymphoreticular and central nervous systems. ...

Prion diseases, or transmissible spongiform encephalopathies, are neurodegenerative diseases, which affect human and many species of animals with 100% fatality rate. The most accepted etiology for prion disease is 'prion', which arises from the conversion from cellular PrP(C) to the pathological PrP(Sc). This review discussed the characteristic structure of PrP, including PRNP gene, PrP(C), PrP...

Prion diseases, also known as the transmissible spongiform encephalopathies (TSEs), are a group of slowly developing neurodegenerations occurring in human and animals. Prion diseases can be transferred between animals, humans, from humans to animals, and from animals to humans. As a result, the central nervous system is attacked, resulting in microglia activation, astrocytosis, prion plaque dep...