Horton W A, Campbell D, Machado M A, Aulthouse A L, Ahmed S, Ellard J T
Department of Pediatrics, University of Texas Medical School, Houston 77225.
Am J Med Genet. 1989 Sep;34(1):91-5. doi: 10.1002/ajmg.1320340116.
As the morphologic expression of the chondrocytic differentiation pathway responsible for bone development and growth, the growth plate has been investigated extensively in the chondrodysplasias. Unique morphologic abnormalities identified in many disorders have provided insight into pathogenetic mechanisms and have been useful diagnostically and nosologically. Biochemical studies have detected evidence of type II collagen defects in patients having disorders in the achondrogenesis type II-spondyloepiphyseal dysplasias (SED) congenita family of chondrodysplasias. Most promising may be the cell culture systems now being developed for human chondrocytes. Preliminary results suggest that they will allow the cellular and molecular biology of the dysplastic growth plate to be directly analyzed.
作为负责骨骼发育和生长的软骨细胞分化途径的形态学表现，生长板在软骨发育不全中已得到广泛研究。在许多疾病中发现的独特形态学异常为发病机制提供了线索，在诊断和分类学上也很有用。生化研究已在属于软骨发育不全的Ⅱ型软骨发育不全-先天性脊柱骨骺发育不良（SED）家族疾病的患者中检测到Ⅱ型胶原蛋白缺陷的证据。最有前景的可能是目前正在为人类软骨细胞开发的细胞培养系统。初步结果表明，它们将使发育异常的生长板的细胞和分子生物学得以直接分析。
