Publication Date:
1990-12-14
Description:
Transgenic mice were created to assess genetic linkage between Gerstmann-Straussler-Scheinker syndrome and a leucine substitution at codon 102 of the human prion protein gene. Spontaneous neurologic disease with spongiform degeneration and gliosis similar to that in mouse scrapie developed at a mean age of 166 days in 35 mice expressing mouse prion protein with the leucine substitution. Thus, many of the clinical and pathological features of Gerstmann-Straussler-Scheinker syndrome are reproduced in transgenic mice containing a prion protein with a single amino acid substitution, illustrating that a neurodegenerative process similar to a human disease can be genetically modeled in animals.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Hsiao, K K -- Scott, M -- Foster, D -- Groth, D F -- DeArmond, S J -- Prusiner, S B -- AG02132/AG/NIA NIH HHS/ -- NS14069/NS/NINDS NIH HHS/ -- NS22786/NS/NINDS NIH HHS/ -- etc. -- New York, N.Y. -- Science. 1990 Dec 14;250(4987):1587-90.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Neurology, University of California, San Francisco 94143.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/1980379" target="_blank"〉PubMed〈/a〉
Keywords:
Amino Acid Sequence
;
Animals
;
Base Sequence
;
Brain/pathology
;
Brain Diseases/*genetics/microbiology/pathology
;
Codon
;
DNA/genetics
;
Disease Models, Animal
;
Endopeptidase K
;
Gerstmann-Straussler-Scheinker Disease/*genetics/microbiology/pathology
;
Leucine
;
Mice
;
Mice, Transgenic
;
Molecular Sequence Data
;
*Mutation
;
Nucleic Acid Hybridization
;
Pedigree
;
PrPSc Proteins
;
Prions/*genetics
;
Serine Endopeptidases/metabolism
;
Transfection
;
Vacuoles/pathology
;
Viral Proteins/*genetics/metabolism
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
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