ASGSB 1998 Annual Meeting Abstracts

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ILLUSTRATIVE DISORDERS OF ECTOPIC SKELETAL MORPHOGENESIS: A CHILDHOOD PARALLAX FOR STUDIES IN GRAVITATIONAL AND SPACE BIOLOGY. Frederick S. Kaplan, M.D.^1,2 and E.M. Shore, Ph.D.^1,3. Departments of ¹Orthopaedic Surgery, ²Medicine, and ³Genetics, The University of Pennsylvania School of Medicine, Philadelphia, PA 19104

Ever since the explosive growth of the animal phyla during the Cambrian radiation nearly 600 million years ago, the evolution of life on Earth has been characterized by gradual changes in body shape and form. Among the most ancient and conserved genes in the animal kingdom are those that regulate morphogenesis. Mutations, or changes, in the genes that control the body plan have lead to the changes in shape and form that we see as the evolutionary process. However, some alterations of these important genes, rather than resulting in the diversity that fills an ecological niche, can cause catastrophic medical problems. Genetic disorders of tissue modeling and morphogenesis provide an important parallax to disturbances of tissue re-modeling that are of paramount importance to gravitational and space biologists as humans begin to explore and live in environments beyond the planet on which they evolved. Disorders of osteogenesis are of particular concern to space biologists due to the dramatic changes in skeletal biology in altered gravitational fields.

Heterotopic ossification is a key feature of at least three distinct genetic disorders of osteogenesis in humans: fibrodysplasia ossificans progressiva, progressive osseous heteroplasia, and Albright's hereditary osteodystrophy. All three conditions are genetic disorders of childhood, but the pathobiology of osteogenic induction, the histopathology of osteogenesis, the anatomic distribution of heterotopic lesions, and the developmental patterns of disease progression differ among the three conditions. The phenotypic distinction of these disorders is critically important in counselling patients and families as well as in advancing research to define the molecular pathophysiology of heterotopic osteogenesis in these disabling genetic disorders. In addition, these disorders of osteogenesis provide an important parallax to disturbances of bone remodeling that are of paramount importance to gravitational and space biologists.

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