The Journal of Bone and Joint Surgery (American). 2009;91:63-67.
doi:10.2106/JBJS.I.00301
© 2009 The Journal of Bone and Joint Surgery, Inc.
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Multiple Hereditary Exostosis and Hedgehog Signaling: Implications for Novel Therapies

Benjamin A. Alman, MD, FRCSC1

1 Division of Orthopaedic Surgery, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada. E-mail address: Benjamin.alman@sickkids.ca

The first 150 words of the full text of this article appear below.


   Introduction
 
Multiple hereditary exostosis is an autosomal dominant condition in which osteochondromas develop at the metaphyseal portions of the bones. In addition, persons with this condition have short stature, asymmetric involvement of the bones, and a small risk of malignant degeneration of an osteochondroma into chondrosarcoma. The location of osteochondromas adjacent to the growth plate suggests that their origin is from growth-plate chondrocytes.

Growth-plate chondrocytes go through a coordinated process of differentiation, resulting in the longitudinal growth of bones. The Indian hedgehog-parathyroid hormone-related protein (Ihh-PTHrP) pathway regulates the rate at which chondrocytes within the growth plate proliferate and differentiate, thus controlling the longitudinal growth of bones. Linkage studies and mutational analysis show that multiple hereditary exostosis is caused by mutations in the EXT genes, the products of which play a role in the diffusion of hedgehog proteins; a mutation in an EXT gene causes abnormal Ihh diffusion, leading to an osteochondroma. . . . [Full Text of this Article]


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