Growth Plate Cartilage Studies in Achondroplasia
Achondroplasia is the most common of the inherited bone dysplasias in man. It is well defined clinically, radiographically and genetically (1, 2). Although its pathogenesis is thought to involve defective endochondral ossification, the specific way in which this occurs is not known. Previous studies of the growth plate in achondroplasia have suggested that the architecture of the growth plate may be mildly abnormal and that there may be abnormalities of extracellular matrix constituents including cartilage collagen and proteoglycan (3, 4). To further explore this issue we have investigated growth plate tissue from 7 patients with heterozygous achondroplasia and 2 patients with homozygous achondroplasia by a combination of biochemical, immunohistochemical, morphometric and ultrastructural methods.
KeywordsGrowth Plate Endochondral Ossification Hypertrophic Chondrocytes Proliferative Zone Cyanogen Bromide
Unable to display preview. Download preview PDF.
- 1.D. L. Rimoin, The chondrodystrophies. Adv. Human Genet. 5:1 (1975).Google Scholar
- 2.W. A. Horton, Bone dysplasias, in: “Practice of Pediatrics”, V. Kelley, ed., Harper & Row, Philadelphia, Chapter 55, pp. 1–30 (1986).Google Scholar
- 3.D. O. Sillence, W. A. Horton and D. L. Rimoin, Morphologic studies in the skeletal dysplasias. Am. J. Pathol. 96:812 (1979).Google Scholar
- 4.A. Pedrini-Mille and V. Pedrini, Proteoglycans and glycosaminoglycans of human achondroplastic cartilage, J. Bone Joint Surg. 64A:39 (1982).Google Scholar
- W. A. Horton and M. A. Machado, Alterations in the extracellular matrix in endochondral ossification in man. Submitted for publication.Google Scholar
- 7.W. A. Horton, J. W. Chou and M. A. Machado, Cartilage collagen analysis in the chondrodystrophies, Collagen Rel. Res. 5:349 (1985).Google Scholar
- 8.O. J. Hood, M. Machado and W. A. Horton, Image analysis of the growth Plate in the study of the skeletal dysplasias. Am. J. Human Genet. 37:A59 (1985).Google Scholar