Introduction: Prion diseases or transmissible spongiform encephalopathies (TSEs) are rare, fatal and incurable neurodegenerative disorders of humans and animals (Prusiner, 1998). In humans, prion diseases occur with unique aetiology as sporadic, genetic or infectious disorders. Sporadic cases of prion diseases, which account for the majority of casualties (up to 85% of all cases), are of unknown origin; the genetic forms are less frequent (up to 15%), while the infectious cases are extremely rare with an incidence of less than 1% (Prusiner, 2001). Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, Fatal Familial Insomnia (FFI) are examples of human prion diseases. In animals the disease is mostly infectious and the mode of transmission is horizontal. Prion diseases include scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle, and chronic wasting disease of deer, elk, and moose (Williams, 2005). The agents responsible for prion diseases are infectious proteins named prions. The term ‘prion’ was coined when Stanley B. Prusiner introduced the concept of proteinaceous infectious particles (Prusiner, 1982). Since the introduction of this once heretical notion, mounting evidence has strengthened its validity. In the next sections of this chapter we present and discuss the peculiar complexity of the molecular pathogenesis of prion diseases in humans and animals.

Molecular pathogenesis of prion diseases / Legname, Giuseppe; Zanusso, G.. - 1:(2012), pp. 95-112. [10.5772/1191]

Molecular pathogenesis of prion diseases

Legname, Giuseppe;
2012-01-01

Abstract

Introduction: Prion diseases or transmissible spongiform encephalopathies (TSEs) are rare, fatal and incurable neurodegenerative disorders of humans and animals (Prusiner, 1998). In humans, prion diseases occur with unique aetiology as sporadic, genetic or infectious disorders. Sporadic cases of prion diseases, which account for the majority of casualties (up to 85% of all cases), are of unknown origin; the genetic forms are less frequent (up to 15%), while the infectious cases are extremely rare with an incidence of less than 1% (Prusiner, 2001). Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, Fatal Familial Insomnia (FFI) are examples of human prion diseases. In animals the disease is mostly infectious and the mode of transmission is horizontal. Prion diseases include scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle, and chronic wasting disease of deer, elk, and moose (Williams, 2005). The agents responsible for prion diseases are infectious proteins named prions. The term ‘prion’ was coined when Stanley B. Prusiner introduced the concept of proteinaceous infectious particles (Prusiner, 1982). Since the introduction of this once heretical notion, mounting evidence has strengthened its validity. In the next sections of this chapter we present and discuss the peculiar complexity of the molecular pathogenesis of prion diseases in humans and animals.
2012
1
Miscellanea on Encephalopathies
95
112
https://www.intechopen.com/books/miscellanea-on-encephalopathies/molecular-pathogenesis-of-prion-diseases
Legname, Giuseppe; Zanusso, G.
File in questo prodotto:
File Dimensione Formato  
36012.pdf

accesso aperto

Tipologia: Versione Editoriale (PDF)
Licenza: Creative commons
Dimensione 473.4 kB
Formato Adobe PDF
473.4 kB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11767/15260
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus ND
  • ???jsp.display-item.citation.isi??? ND
social impact