Fibrillin network in normal bone and cartilage

The molecular basis for Marfan's syndrome is known to reside in mutations i n FBN1, the gene for fibrillin 1. The skeletal manifestations of Marfan syn drome include morphologic abnormalities and osteopenia. Presence and distri bution of fibrillin 1 in adult bone (healthy or with Marfan syndrome) has n ot been studied extensively. We evaluated distribution of fibrillin and typ e III collagen in bone and cartilage of children and adults without bone di sease, using monoclonal antibodies. Fibrillin is mostly present in attachme nt sites for tendons. In cartilage and bone tissue, fibrillin is identified at the junction between cartilage and bone in children, and in the areas w ith intense osteoblastic activity. These data suggest participation of fibrillin in bone formation and growth during youth and in bone mineralisation in adult.