Growth plate cartilage studies in achondroplasia.
Growth plate cartilage studies in achondroplasia.
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软骨发育不全的生长板软骨研究。
DOI：
10.1007/978-1-4684-8712-1_11
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发表时间：
1988
期刊：
影响因子：
--
通讯作者：
Campbell,D
中科院分区：
文献类型：
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作者：
Horton,WA;Hood,OJ;Machado,MA;Campbell,D
软骨发育不全是人类最常见的遗传性骨发育不良，在临床、影像学和遗传学上都有明确的定义（1，2）。尽管其发病机制被认为与软骨内成骨缺陷有关，但其发生的具体方式尚不清楚。先前对软骨发育不全中生长板的研究表明，生长板的结构可能轻度异常，并且可能存在细胞外基质成分（包括软骨胶原和蛋白聚糖）的异常（3，4）。为了进一步探讨这个问题，我们研究了生长板组织从7例杂合型软骨发育不全和2例纯合型软骨发育不全的生物化学，免疫组化，形态计量学和超微结构的方法相结合。
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.