Bonucci E, Marco A D, Nicoletti B, Petrinelli P, Pozzi L
Growth. 1976 Sep;40(3):241-51.
Histological and histochemical investigations of tibial epiphyses, costo-chondral junctions and caudal vertebrae of achondroplastic (cn) mice have shown that, in spite of conspicuous reduction of bone length, endochondrial ossification occurs in much the same way as in controls. Moreover, the histological structure of seriated cartilage, and the distribution of proteoglycans in resting cartilage are the same in cn/cn mice and in controls. The only difference betweeen the two types of animals is represented by the occurence of early aging-like changes of chondrocytes and cartilage matrix in achondroplastic mice, leading to premature shortening of the cell columns and to early reduction of the proteoglycan concentration. The premature "aging" of the cartilage, the consequent inhibition of the calcification process and bone growth, and the normal rate of perichondral ossification give to long bones, vertebrae and ribs a typical achondroplastic appearance. The cn/cn mice seem to represent a useful model for studying the pathogenesis and therapy of human achondroplasia.
对软骨发育不全（cn）小鼠的胫骨骨骺、肋软骨结合处和尾椎进行的组织学和组织化学研究表明，尽管骨长度明显缩短，但软骨内成骨的发生方式与对照组基本相同。此外，cn/cn小鼠和对照组中，连续软骨的组织结构以及蛋白聚糖在静止软骨中的分布是相同的。这两种动物之间的唯一差异表现为软骨发育不全小鼠的软骨细胞和软骨基质出现类似早期衰老的变化，导致细胞柱过早缩短以及蛋白聚糖浓度过早降低。软骨的过早“老化”、随之而来的钙化过程和骨生长的抑制，以及软骨膜成骨的正常速率，使长骨、椎骨和肋骨呈现出典型的软骨发育不全外观。cn/cn小鼠似乎是研究人类软骨发育不全发病机制和治疗方法的有用模型。
