Kikukawa K, Kamei T, Suzuki K
Department of Veterinary Physiology, Nippon Veterinary and Zootechnical College, Tokyo, Japan.
Connect Tissue Res. 1991;25(3-4):301-9. doi: 10.3109/03008209109029165.
The osteochondrodysplasia rat, inherited by a single autosomal recessive lethal gene ocd, shows a typical dwarfing syndrome with systemic subcutaneous edema. The skeletal system is most severely affected. The affected neonates are associated with cleft palate, abnormal kidney position and central nerve system malfunction. The present study describes histological and histochemical appearances of the affected epiphysial cartilage. Irregular columnization, thinner hypertrophic zone, swelled chondrocytes, tightly packed chondrocytes with a poor amount of cartilage matrix was found in the affected. The defining characteristic was a wide-spread degenerating area from the resting to hypertrophic zone. The extracellular matrix (ECM) reacted weakly for the glycosaminoglycans (GAGs). A reduced content of sialic acid in the ECM was suggested. It is concluded that the cartilage abnormalities in the ocd/ocd is a new type disease of osteochondrodysplasia possibly due to some defects in GAGs and/or sialic acid metabolism.
骨软骨发育不良大鼠由单个常染色体隐性致死基因ocd遗传，表现出典型的侏儒综合征并伴有全身性皮下水肿。骨骼系统受影响最为严重。患病新生儿伴有腭裂、肾脏位置异常和中枢神经系统功能障碍。本研究描述了患病骺软骨的组织学和组织化学表现。在患病软骨中发现细胞柱排列不规则、肥大带变薄、软骨细胞肿胀、软骨细胞紧密堆积且软骨基质数量稀少。其决定性特征是从静止区到肥大区广泛的退变区域。细胞外基质（ECM）对糖胺聚糖（GAGs）反应较弱。提示ECM中唾液酸含量降低。结论是ocd/ocd中的软骨异常是一种新型的骨软骨发育不良疾病，可能是由于GAGs和/或唾液酸代谢存在某些缺陷所致。
