Fibrillins are large extracellular macromolecules that polymerize to form the backbone structure of connective tissue microfibrils. Mutations in the gene for fibrillin-1 cause the Marfan syndrome. while mutations in the gene for fibrillin-2 cause Congenital Contractural Arachnodactyly. Both are autosomal dominant disorders. https://www.roneverhart.com/Deauville-by-Michel-Germain/
Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice.
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