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
MissingFormLabel
- 2
Prusiner S B.
Prions.
Proc Natl Acad
Sci.
1998;
95
13 363-13 383
MissingFormLabel
- 3
Prusiner S B.
Molecular biology of prion
diseases.
Science.
1991;
252
1515-1522
MissingFormLabel
- 4
Harris D A.
Cellular biology of prion diseases.
Clin Microbiol
Rev.
1999;
12
429-444
MissingFormLabel
- 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
MissingFormLabel
- 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
MissingFormLabel
- 7
Collee J G, Bradley R.
BSE: A decade on-part
I.
Lancet.
1997;
349
636-641
MissingFormLabel
- 8
Ghani A C, Ferguson N M, Donnelly C A, Anderson R M.
Predicted vCJD mortality in Great
Britain.
Nature.
2000;
406
583-584
MissingFormLabel
- 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
MissingFormLabel
- 10
Cohen F E, Prusiner S B.
Pathologic conformations of prion proteins.
Ann Rev
Biochem.
1998;
67
793-819
MissingFormLabel
- 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
MissingFormLabel
- 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
MissingFormLabel
- 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
MissingFormLabel
- 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
MissingFormLabel
- 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
MissingFormLabel
- 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
MissingFormLabel
- 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
MissingFormLabel
- 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
MissingFormLabel
Prof. S. Ghosh
Gastroenterology Section, Imperial College School of
Medicine
Hammersmith Campus
London W12 0NN
Email: s.ghosh@ic.ac.uk