Phenotype Image Detail

Image

Caption

Tg(Prnp*S170N*N174T)1020Aag/0 Prnp^tm1Cwe/Prnp^tm1Cwe 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 Prnp^tm1Cwe/Prnp^tm1Cwe (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
Prnp^tm1Cwe	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
Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp*S170N*N174T)1020Aag/0	involves: 129S7/SvEvBrd * C57BL/6