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Phenotype Image Detail
Image
Caption A: (Left two panels) Representative aneurysm of the ascending aorta in a newborn mouse with histological documentation (Weigert staining) of elastic fiber fragmentation and aortic wall rupture (arrow). (Right four panels) Top, right lungs of wild type (+/+) and Fbn1tm3Rmz/Fbn1tm3Rmz (-/-) mice showing multiple blebs in the peripheral portion of the latter (arrow); bottom, partial views of the exposed thoraxes of wild type and mutant mice showing thinner intercostal muscles and abnormal ribs in the mutant compared with the wild type. B: Cross section of aortic tissue around the lesions in mutant mice and at corresponding levels in age and sex-matched wild type littermates stained with hematoxylin and eosin, Weigert, trichrome, or anti-MMP-9 antiserum. On the right side of the H & E panels is a magnification of the mutant tissue showing an altered architecture with loss of normal contacts between cells and with the surrounding matrix (yellow arrows). The immunoblot on the right further documents elevated MMP-9 compared with beta-tubulin levels in the decaying post-mortem aorta of mutant mice. All the specimens are oriented with the lumen of the vessel at the top. Scale bar, 100um.
Copyright This image is from Carta L, J Biol Chem 2006 Mar 24;281(12):8016-23 and is displayed with the permission of the American Society for Biochemistry and Molecular Biology who owns the Copyright. Full text from JBC. J:110586
Associated
Alleles
Symbol Name
Fbn1tm3Rmz fibrillin 1; targeted mutation 3, Francesco Ramirez
Associated
Genotypes
Allelic Composition Genetic Background
Fbn1tm3Rmz/Fbn1tm3Rmz Not Specified

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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
12/16/2014
MGI 5.20
The Jackson Laboratory