Prion diseases are invariably fatal neurodegenerative disorders affecting humans and many mammals. Here, we will discuss the current understanding of prion biology and the molecular biology of this group of illnesses. We introduce several aspects of prion biology, from the primary structure of the cellular form of the prion protein, PrPC, to the conformational changes that occur during the conversion to the pathological form, PrPSc. In particular, we provide recent developments in our understanding of the molecular determinants of prion infectivity. We present in detail the discovery of synthetic mammalian prions and the implications that such findings may have for the future of prion research. © 2012 by Nova Science Publishers, Inc. All rights reserved.
Synthetic prions
	
	
	
		
		
		
		
		
	
	
	
	
	
	
	
	
		
		
		
		
		
			
			
			
		
		
		
		
			
			
				
				
					
					
					
					
						
						
							
							
						
					
				
				
				
				
				
				
				
				
				
				
				
			
			
		
			
			
				
				
					
					
					
					
						
						
							
							
						
					
				
				
				
				
				
				
				
				
				
				
				
			
			
		
			
			
				
				
					
					
					
					
						
							
						
						
					
				
				
				
				
				
				
				
				
				
				
				
			
			
		
		
		
		
	
Giachin G.
	
		
		
	
			2012
Abstract
Prion diseases are invariably fatal neurodegenerative disorders affecting humans and many mammals. Here, we will discuss the current understanding of prion biology and the molecular biology of this group of illnesses. We introduce several aspects of prion biology, from the primary structure of the cellular form of the prion protein, PrPC, to the conformational changes that occur during the conversion to the pathological form, PrPSc. In particular, we provide recent developments in our understanding of the molecular determinants of prion infectivity. We present in detail the discovery of synthetic mammalian prions and the implications that such findings may have for the future of prion research. © 2012 by Nova Science Publishers, Inc. All rights reserved.Pubblicazioni consigliate
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