Article
Forelimb contractures and abnormal tendon collagen fibrillogenesis in fibulin-4 null mice.
Cell and tissue research - 1 Jun 2016
Markova Dessislava Z, Pan Te-Cheng, Zhang Rui-Zhu, Zhang Guiyun, Sasaki Takako, Arita Machiko, Birk David E, Chu Mon-Li
Abstract excerpt
Fibulin-4 is an extracellular matrix glycoprotein essential for elastic fiber formation. Mice deficient in fibulin-4 die perinatally because of severe pulmonary and vascular defects associated with the lack of intact elastic fibers. Patients with fibulin-4 mutations demonstrate similar defects, and a significant number die shortly after birth or in early childhood from cardiopulmonary failure. The patients also...
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