Characterization of Cardiac Function and Arterial Mechanics During Early Postnatal Development in Fibulin-5 Null Mice
2013 ◽
Keyword(s):
Fibulin-5 is an extracellular matrix protein that interacts with other proteins during a complex process that results in elastic fiber formation from the elastin precursor, tropoelastin [1]. Elastic fibers are an important component of tissues requiring elasticity, including large arteries, lungs and skin. In mice lacking fibulin-5 ( Fbln5−/−), these tissues contain disorganized elastic fibers and exhibit decreased elasticity [2]. The phenotype of Fbln5−/− mice is similar to that of humans with cutis laxa, a connective tissue disorder characterized by loose skin and narrow arteries with reduced compliance.
2007 ◽
Vol 28
(3)
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pp. 1061-1067
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Keyword(s):
2021 ◽
2017 ◽
Vol 313
(4)
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pp. L687-L698
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1985 ◽
Vol 4
(9)
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pp. 2145-2151
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2017 ◽
Vol 114
(22)
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pp. E4408-E4415
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2018 ◽
Vol 89
(5)
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pp. 352-361
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