Transmissible Spongiform Encephalopathies (TSEs)

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Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. The proteinaceous infectious particles, prion was identified by an American neurologist Stanley B. Prusiner and colleagues in 1982derived from the words protein and infection (Stanley B. Prusiner -Autobiography). Prions are known to Creutzfeldt - Jakob disease (CJD), Gerstmann–Sträussler–Scheinker syndrome and Fatal Familiar Insomnia in humans. In mammals they cause "mad cow disease" in cattle. Prion diseases affect the structure of the brain or other neural tissue and all are currently universally fatal and untreatable (Prusiner, 1998).
Transmissible spongiform encephalopathies (TSEs) are caused by abnormal folding of prions. The normal prion protein, which is designated as PrPc, is a 35kD membrane glycoprotein, which is water-soluble and proteinase-sensitive(Laurén, 2009).Abnormal prions, designated as PrPSc or PrPTSE, result from a change in the folding pattern of PrPc, which makes it resistant to the action of proteases and causes it to precipitate as insoluble amyloid (Robbins, 1999). This conversion results in neuronal degeneration and loss by an unknown mechanism. PrPc is encoded by the PRNP gene on chromosome 20 and probably plays a role in multiple cellular functions, including cell adhesion, ion channel activity, and neuronal excitability (Lindquist, 2001).
PrPC protein structure is composed of a normal protein found on the cell membranes. It has been reported that PrP play significant roles in intr...

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..., suggesting that direct cell proximity promoted efficient infection. The fact that living cells were far more effective in transmitting infectivity than dead cells argues that cell biological processes are involved in prion transmission (MacKenzie, 2011).
Potential treatments and diagnosis
Biological are accounting for an ever increasing fraction of all therapeutics—yet all eukaryotically produced biological bear a certain risk of prion contamination, even when generated in cell lines (Brown, 2003). The transmission of CJD through blood and even purified blood products has dramatically highlighted the seriousness of this threat. Therefore, PrPC-deficient farm animals (cattle and goats) are well positioned for the production of prion-free therapeutics and will therefore make an important contribution towards eliminating the risk of prions contamination in biological

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