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Congenital skeletal dysplasias — a better understanding via experimental models

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Teratogenic Mechanisms

Part of the book series: Advances in the Study of Birth Defects ((ASBD,volume 1))

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Abstract

The two processes involved in bone formation are ossification and calcification. Ossification is the production of bone matrix in which the precipitation of minerals occurs (calcification). Chondrocytes, and more so osteoblasts, are primarily involved in both processes. During life (both during the active period of growth and after cessation of this process) there is a continuous process of bone remodelling — formation and resorption of bone, a dynamic process which causes growth of bone, fracture repair and maintenance of the proper skeletal configuration1. Different enzymes are involved in the processes of bone formation, growth and remodelling, and failure of one of these enzymes may cause failure of skeletal growth, thus producing distinct abnormalities — skeletal dysplasias. Although enzymopathies are the regular cause of skeletal dysplasias, and the missing enzymes are usually not well-defined, the biochemical deviation observed may also sometimes not be the primary cause, but rather the results of the skeletal defect2.

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  1. A. Ornoy
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Editors and Affiliations

  1. Department of Anatomy, Faculties of Medicine and Dentistry, University of Manitoba, 730 William Avenue, R3E 0W3, Winnipeg, Manitoba, Canada

    T. V. N. Persaud

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Ornoy, A. (1979). Congenital skeletal dysplasias — a better understanding via experimental models. In: Persaud, T.V.N. (eds) Teratogenic Mechanisms. Advances in the Study of Birth Defects, vol 1. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-5910-4_4

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  • DOI: https://doi.org/10.1007/978-94-011-5910-4_4

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-011-5912-8

  • Online ISBN: 978-94-011-5910-4

  • eBook Packages: Springer Book Archive

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