Phenotype Image Detail

Image

Caption

Phenotype of Grn^tm2.1Far/Grn^tm2.1Far (Grn^R493X/R493X) mice recapitulates features of global knockout mice. (A) Mutant mice exhibit age-dependent microgliosis. Immunostaining of Iba1 in the thalamus. Arrowheads indicate selected Iba1+ cells. (C and D) Increased levels of total (C) and phosphorylated (D) TDP-43 in the cytoplasm of thalamic neurons of 12-month-old Grn^tm2.1Far/Grn^tm2.1Far and Grn^tm1.1Far/Grn^tm1.1Far (Grn^-/-) mice. Arrowheads indicate cytoplasmic accumulation of TDP-43. (Scale bars, 20 um.) (E) Grn^tm2.1Far/Grn^tm2.1Far mice exhibit age-dependent reduction of synaptic density. (E) Immunostaining of synaptophysin in the thalamus of 13-month-old mice. (Scale bar, 10 um.) (G) Grn^tm2.1Far/Grn^tm2.1Far mutant mice have increased lipofuscin in the brain. (G) Images of autofluorescent lipofuscin (green channel) in the thalamus of 19-month-old mice. (Scale bar, 10 um.) (I) Grn^tm2.1Far/Grn^tm2.1Far mutant mice have increased skin lesions resulting in decreased survival. (I) Skin lesions in 16-month old mice.

Copyright

This image is from Nguyen AD, Proc Natl Acad Sci U S A 2018 Mar 20;115(12):E2849-E2858. Copyright 2018 National Academy of Sciences, U.S.A. J:259651

Associated Alleles

Symbol	Name
Grn^tm2.1Far	granulin; targeted mutation 2.1, Bob Farese

Associated Genotypes

Allelic Composition	Genetic Background
Grn^tm2.1Far/Grn^tm2.1Far	involves: 129S4/SvJae * C57BL/6