Article
Fibrillin microfibrils in bone physiology.
Matrix biology : journal of the International Society for Matrix Biology - 1 Jan 2000
Smaldone Silvia, Ramirez Francesco
Abstract excerpt
The severe skeletal abnormalities associated with Marfan syndrome (MFS) and congenital contractural arachnodactyly (CCA) underscore the notion that fibrillin assemblies (microfibrils and elastic fibers) play a critical role in bone formation and function in spite of representing a low abundance component of skeletal matrices. Studies of MFS and CCA mice have correlated the skeletal phenotypes of these mutant...
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