Subscribe to RSS
DOI: 10.1055/s-2002-19641
Intestinal Entry of Prions
Prioneneintritt über den DünndarmPublication History
                     4.10.2001
                     
                     4.10.2001
                     
Publication Date:
21 January 2002 (online)

Abstract
Variant Creutzfeldt-Jakob disease is thought to be caused by infected prion protein via ingestion of contaminated beef. After ingestion of infected prion proteins, uptake by small intestine may be by either M-cell dependent or M-cell independent routes. A receptor for prion protein, laminin receptor precursor is expressed on the brush border of small intestinal epithelium in 40 % of subjects. The cellular prion protein expressed on the enteric nervous system might serve as the target for conversion to infectious prion protein and a route for spread to the central nervous system.
Zusammenfassung
Eine Variante der Creutzfeldt-Jakob-Krankheit soll über die Aufnahme kontaminierten Rindfleischs durch infektiöses Prionenprotein hervorgerufen werden. Nach dem Verzehr können die Prionenproteine im Dünndarm durch Vermittlung der M-Zellen oder unabhängig davon aufgenommen werden. Bei 40 % aller Menschen wird am Bürstensaum des Dünndarmepithels ein Rezeptor für Prionenproteine, der Lamininrezeptor, exprimiert. Das im enterischen Nervensystem exprimierte zelluläre Prionenprotein könnte als Ziel für die Umwandlung in infektiöses Protein dienen und den Weg zur Ausbreitung ins Zentralnervensystem darstellen.
Key words
Prions - Variant CJD - Laminin Receptor Protein - Small Intestine
Schlüsselwörter
Prionen - CJD-Variante - Laminin-Rezeptorprotein - Dünndarm
References
- 1 
            Prusiner S B. 
            Novel proteinaceous infectious particles cause 
            					 scrapie. 
            Science. 
            1982; 
            216 
            136-144 
            
            Reference Ris Wihthout Link
- 2 
            Prusiner S B. 
            Prions. 
            Proc Natl Acad 
            					 Sci. 
            1998; 
            95 
            13 363-13 383 
            
            Reference Ris Wihthout Link
- 3 
            Prusiner S B. 
            Molecular biology of prion 
            					 diseases. 
            Science. 
            1991; 
            252 
            1515-1522 
            
            Reference Ris Wihthout Link
- 4 
            Harris D A. 
            Cellular biology of prion diseases. 
            Clin Microbiol 
            					 Rev. 
            1999; 
            12 
            429-444 
            
            Reference Ris Wihthout Link
- 5 
            Pan K M, Baldwin M, Nguyen J 
            						  . et al .
            Conversion of alpha-helices into β-sheets features in 
            					 the formation of scrapie prion proteins. 
            Proc Natl Acad 
            					 Sci. 
            1993; 
            90 
            10 962-10 966 
            
            Reference Ris Wihthout Link
- 6 
            Will R G, Ironside J W, Zeidler M 
            						  . et al .
            A new-variant of Creutzfeldt- Jakob disease in the 
            					 UK. 
            Lancet. 
            1996; 
            347 
            921-925 
            
            Reference Ris Wihthout Link
- 7 
            Collee J G, Bradley R. 
            BSE: A decade on-part 
            					 I. 
            Lancet. 
            1997; 
            349 
            636-641 
            
            Reference Ris Wihthout Link
- 8 
            Ghani A C, Ferguson N M, Donnelly C A, Anderson R M. 
            Predicted vCJD mortality in Great 
            					 Britain. 
            Nature. 
            2000; 
            406 
            583-584 
            
            Reference Ris Wihthout Link
- 9 
            Cousens S, Smith P G, Ward H 
            						  . et al .
            Geographical distribution of variant Creutzfeldt-Jakob 
            					 disease in Great Britain, 
            					 1996-2000. 
            Lancet. 
            2001; 
            357 
            1002-1007 
            
            Reference Ris Wihthout Link
- 10 
            Cohen F E, Prusiner S B. 
            Pathologic conformations of prion proteins. 
            Ann Rev 
            					 Biochem. 
            1998; 
            67 
            793-819 
            
            Reference Ris Wihthout Link
- 11 
            Beekes M, McBride P A. 
            Early accumulation of pathological PrP in the enteric nervous 
            					 system and gut-associated lymphoid tissue of hamsters orally infected with 
            					 scrapie. 
            Neurosci 
            					 Lett. 
            2000; 
            278 
            181-184 
            
            Reference Ris Wihthout Link
- 12 
            Rieger R, Edenhofer F, Lasmezas C I, Weiss S. 
            The human 37-kDa laminin receptor precursor interacts with 
            					 the prion protein in eukaryotic cells. 
            Nat 
            					 Med. 
            1997; 
            3 
            1383-1388 
            
            Reference Ris Wihthout Link
- 13 
            Shmakov A N, Bode J, Kilshaw P J, Ghosh S. 
            Diverse patterns of expression of the 67-kD laminin receptor 
            					 in human small intestinal mucosa: Potential binding sites for prion 
            					 proteins. 
            J 
            					 Pathol. 
            2000; 
            191 
            318-322 
            
            Reference Ris Wihthout Link
- 14 
            Shmakov A N, McLennan N F, McBride P 
            						  . et al .
            Cellular prion protein is expressed in the human enteric 
            					 nervous system. 
            Nat 
            					 Med. 
            2000; 
            6 
            840-841 
            
            Reference Ris Wihthout Link
- 15 
            Wadsworth J DF, Joiner S, Hill A F 
            						  . et al .
            Tissue distribution of protease resistant prion protein in 
            					 variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting 
            					 assay. 
            Lancet. 
            2001; 
            358 
            171-180 
            
            Reference Ris Wihthout Link
- 16 
            Ironside J W, Head M W, Bell J E, McCardle L, Will R G. 
            Laboratory diagnosis of variant Creutzfeldt-Jakob 
            					 disease. 
            Histopathology. 
            2000; 
            37 
            1-9 
            
            Reference Ris Wihthout Link
- 17 
            Hilton D A, Fathers E, Edwards P, Ironside J W, Zajicek J. 
            Prion immunoreactivity in appendix before clinical onset of 
            					 variant Creutzfeldt-Jakob 
            					 disease. 
            Lancet. 
            1998; 
            352 
            703-704 
            
            Reference Ris Wihthout Link
- 18 
            Bruce M E, McConnell I, Will R G, Ironside J W. 
            Detection of variant Creutzfeldt-Jakob disease infectivity in 
            					 extraneural 
            					 tissues. 
            Lancet. 
            2001; 
            358 
            208-209 
            
            Reference Ris Wihthout Link
Prof. S. Ghosh
         Gastroenterology Section, Imperial College School of 
         			 Medicine
         
         Hammersmith Campus
         
         London W12 0NN
         
         Email: s.ghosh@ic.ac.uk
         
         
 
     
      
    