Phenotype Image Detail

Image

Caption

Proteinase K resistant prion protein (PrP^res) and prion protein (PrP) aggregates in Prnp^tm2Lnq/Prnp^tm2Lnq (FFI) and Prnp^tm3Lnq/Prnp^tm3Lnq (CJD) brains. (B) Serial sections of a cerebellar folium were incubated in PBS solution with or without Proteinase K (PK+ or PK-), followed by immunostaining for antibodies against the N or C terminus of PrP (SAF32 or SAF84, respectively). Aggregates in CJD mice are composed of PrP that is reactive with SAF32, indicating that PrP containing the N terminus (and likely full length) is aggregating. The N terminus is sensitive to PK, like the PrPres detected in human prion diseases and mouse models of acquired prion diseases. (C-E) PrP deposits in FFI mice. (C) An example of a deposit in a FFI thalamus, revealed by standard EM, is framed by four white asterisks. (D and E) An example of a deposit in a FFI thalamus, revealed by immuno-EM using mild fixation. (E) Higher magnification of the area marked in D with the black rectangle. Arrowheads in E mark immunogold particles. (Scale bars: C and D, 500 nm; E, 62.5 nm.)

Copyright

This image is from Jackson WS, Proc Natl Acad Sci U S A 2013 Sep 3;110(36):14759-64. Copyright 2013 National Academy of Sciences, U.S.A. J:200974

Associated Alleles

Symbol	Name
Prnp^tm2Lnq	prion protein; targeted mutation 2, Susan Lindquist
Prnp^tm3Lnq	prion protein; targeted mutation 3, Susan Lindquist

Associated Genotypes

Allelic Composition	Genetic Background
Prnp^tm2Lnq/Prnp^tm2Lnq	involves: 129P2/OlaHsd * C57BL/6N
Prnp^tm3Lnq/Prnp^tm3Lnq	involves: 129P2/OlaHsd * C57BL/6N