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Phenotype Image Detail
Image
Caption Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+ Tg(Tagln-cre)1Her/0 (R1031C) and Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Gt(ROSA)26Sor+ Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg Tg(Tagln-cre)1Her/0 (C455R) transgenic mice develop vascular smooth muscle cell (vSMC) abnormalities. (A-D) Representative electron micrographs (EM) from 12- to 14-month-old (A-C) (n = 2) or 19- to 21-month-old (D) (n = 2) animals carrying the human R1031C mutation in a mouse Notch 3 wild-type (WT) background. Morphological abnormalities were absent in animals carrying the R1031C mutation at 6 months of age (n = 2). (E and F) Representative EM images from 6-month-old animals carrying the C455R mutation in a Notch 3 KO background (n = 2). (A) Overview of a vessel with abundant elastin and an electron-dense granular deposit (arrow). (B) Accumulation of extracellular granular material (arrow) and cytoplasmic inclusions (asterisk). (C) An almost occluded vessel with accumulations of granular material. (D) Accumulation of granular material and inclusions (arrow). (E) Accumulation of electron-dense granular material (arrows). (F) Inclusions (asterisk) and electron-dense granular material (arrow). The brain tissue analyzed was compared with tissue from age-matched controls expressing the WT NOTCH 3 (n = 5) and from animals that lacked the Tg(Tagln-cre)1Her (SM22-Cre driver) (n = 5) processed in parallel. L, lumen. (Scale bars: A, 2 um; B and C, 1 um; D and F, 2 um; E, 500 nm.)
Copyright This image is from Arboleda-Velasquez JF, Proc Natl Acad Sci U S A 2011 May 24;108(21):E128-35. Copyright 2011 National Academy of Sciences, U.S.A. J:171887
Associated
Alleles
Symbol Name
Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat gene trap ROSA 26, Philippe Soriano; targeted mutation 2, Spyros Artavanis-Tsakonas
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat gene trap ROSA 26, Philippe Soriano; targeted mutation 3, Spyros Artavanis-Tsakonas
Notch3Gt(PST033)Byg notch 3; gene trap PST033, BayGenomics
Tg(Tagln-cre)1Her transgene insertion 1, Joachim Herz
Associated
Genotypes
Allelic Composition Genetic Background
Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0 		involves: 129 * C57BL/6 * SJL
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+
Tg(Tagln-cre)1Her/0 		involves: 129S2/SvPas * 129S6/SvEvTac * C57BL/6 * SJL
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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04/23/2024
MGI 6.23 		The Jackson Laboratory