Wikström B, Hjerpe A, Reinholt F P, Engfeldt B
Coll Relat Res. 1986 Jul;6(3):279-93. doi: 10.1016/s0174-173x(86)80012-7.
The achondroplastic mouse is a dwarf mouse in which the endochondral growth of the skeleton is disturbed. The main morphological characteristic of the affected growth cartilage is an underdeveloped hypertrophic zone. The pattern of matrix mineralization seems to be unaffected, even in areas where hypertrophic chondrocytes are completely absent. The present stereological results indicate a disturbance of the cn/cn cartilage already in the proliferative zone. At the electron microscopical level a clearly abnormal spatial distribution of matrix vesicles was observed in the affected growth cartilage. The vesicle concentration of the cn/cn cartilage was increased, but the mineralizing cartilage showed a decrease of vesicles similar to that in the normal tissue. Parallel biochemical characterization of the proteoglycans and glycosaminoglycans of the cn/cn costal cartilage revealed normal conditions, also in the growth region, which was sampled as a separate tissue fraction. Thus the biochemical results provide no evidence of disturbed glycosaminoglycan metabolism.
软骨发育不全小鼠是一种骨骼软骨内生长受到干扰的侏儒小鼠。受影响的生长软骨的主要形态学特征是肥大带发育不全。基质矿化模式似乎未受影响，即使在完全没有肥大软骨细胞的区域也是如此。目前的体视学结果表明，在增殖区，cn/cn软骨就已受到干扰。在电子显微镜水平上，在受影响的生长软骨中观察到基质小泡的空间分布明显异常。cn/cn软骨的小泡浓度增加，但矿化软骨的小泡数量减少，与正常组织相似。对cn/cn肋软骨的蛋白聚糖和糖胺聚糖进行的平行生化分析表明，即使在作为单独组织部分取样的生长区域，情况也是正常的。因此，生化结果没有提供糖胺聚糖代谢受到干扰的证据。
