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Overexpression of an Osteogenic Morphogen in Fibrodysplasia Ossificans Progressiva

1996; Massachusetts Medical Society; Volume: 335; Issue: 8 Linguagem: Inglês

10.1056/nejm199608223350804

1533-4406

Adam B. Shafritz, Eileen M. Shore, Francis H. Gannon, Michael Zasloff, Rebecca Taub, Maximilian Muenke, Frederick S. Kaplan,

Bone Tumor Diagnosis and Treatments

Fibrodysplasia ossificans progressiva is a heritable disorder of connective tissue characterized by congenital malformation of the great toes and postnatal formation of ectopic bone. Although the disorder was first described more than 300 years ago, the genetic defect and pathophysiology remain unknown. Bone morphogenetic proteins are potent bone-inducing morphogens that participate in the developmental organization of the skeleton, and increased production of one or more of these proteins has been proposed as the cause of fibrodysplasia ossificans progressiva.