Article
Marfan-like skeletal phenotype in the tight skin (Tsk) mouse.
Calcified tissue international - 1 Oct 2007
Barisic-Dujmovic Tatjana, Boban Ivana, Adams Douglas J, Clark Stephen H
Abstract excerpt
Tight skin (Tsk) is an autosomal dominant mutation located on mouse chromosome 2 and is associated with an intragenic duplication of the fibrillin 1 (Fbn1) gene. Mutant mice (Tsk/+) display a tightness of skin in the interscapular region, lung emphysema, myocardial hypertrophy, skeletal overgrowth, and kyphosis. It is hypothesized in this study that in Tsk mice the mutation in Fbn1 alters bone cell metabolism. A...
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