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Phenotype Image Detail
Image
Caption Tg(Prnp*S170N*N174T)1020Aag/0 Prnptm1Cwe/Prnptm1Cwe mice develop a lethal neurologic disease with lesions in the brain and muscle. (A) Clinically healthy Tg(Prnp)a20Cwe/0 (tga20) mouse (Left) and Tg(Prnp*S170N*N174T)1020Aag/0 Prnptm1Cwe/Prnptm1Cwe (tg1020) mouse showing kyphosis and paraparesis, with hind legs tucked under the body. C) Hippocampi of tg1020 and tga20 mice. Arrows indicate spongiform change. Linear arrays of PrP aggregates were accompanied by severe gliosis and microglial activation, as detected by antibodies to PrP, GFAP, and Iba1, respectively. Asterisk indicates a small vessel in a section of a tga20 brain. (D) Myopathy in tg1020 but not tga20 mice, with centralized nuclei (arrowhead), split fibers (arrow), and hypervariable fiber calibers. PrP immunostains show deposits (arrow) in affected tg1020 muscles.
Copyright This image is from Sigurdson CJ, Proc Natl Acad Sci U S A 2009 Jan 6;106(1):304-9. Copyright 2009 National Academy of Sciences, U.S.A. J:143528
Associated
Alleles
Symbol Name
Prnptm1Cwe prion protein; targeted mutation 1, Charles Weissmann
Tg(Prnp)a20Cwe transgene insertion a20, Charles Weissmann
Tg(Prnp*S170N*N174T)1020Aag transgene insertion 1020, Adriano Aguzzi
Associated
Genotypes
Allelic Composition Genetic Background
Tg(Prnp)a20Cwe/0 Not Specified
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp*S170N*N174T)1020Aag/0
involves: 129S7/SvEvBrd * C57BL/6

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
04/30/2024
MGI 6.23
The Jackson Laboratory