Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein

PrP c is a host protein anchored to the outer surface of neurons and to a lesser extent of lymphocytes and other cells. The transmissible agent (prion) responsible for scrapie is believed to be a modified form of PrP c . Mice homozygous for disrupted PrP genes have been generated. Surprisingly, they...

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Bibliographic Details
Published in:Nature (London) 1992-04, Vol.356 (6370), p.577-582
Main Authors: Büeler, Hansruedi, Fischer, Marek, Lang, Yolande, Bluethmann, Horst, Lipp, Hans-Peter, DeArmond, Stephen J, Prusiner, Stanley B, Aguet, Michel, Weissmann, Charles
Format: Article
Language:English
Subjects:
Aging
Animals
Base Sequence
Biological and medical sciences
Blastocyst - physiology
Blotting, Northern
Blotting, Western
Brain - growth & development
Brain - physiology
Cell Membrane - physiology
Cellular biology
Crosses, Genetic
Female
Genetic Carrier Screening
Humanities and Social Sciences
Lymphocytes
Male
Medical research
Medical sciences
membrane proteins
Membrane Proteins - genetics
Mice
Molecular Sequence Data
multidisciplinary
Nervous system involvement in other diseases. Miscellaneous
Neurological disorders
Neurology
Neurons - physiology
Oligodeoxyribonucleotides
Polymerase Chain Reaction
Pregnancy
prion protein
Prions - genetics
Proteins
PrPSc Proteins
Recombination, Genetic
Restriction Mapping
RNA - genetics
RNA - isolation & purification
Science
Science (multidisciplinary)
scrapie
Scrapie - genetics
Stem Cells - physiology
Viruses
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Description
Summary:PrP c is a host protein anchored to the outer surface of neurons and to a lesser extent of lymphocytes and other cells. The transmissible agent (prion) responsible for scrapie is believed to be a modified form of PrP c . Mice homozygous for disrupted PrP genes have been generated. Surprisingly, they develop and behave normally for at least seven months, and no immunological defects are apparent. It is now feasible to determine whether mice devoid of PrP c can propagate prions and are susceptible to scrapie pathogenesis.
ISSN:0028-0836
1476-4687
DOI:10.1038/356577a0