Silberberg R, Lesker P
Growth. 1975 Mar;39(1):17-33.
Skeletal development of achondroplastic (cn/cn) mice, aged 2 to 7 weeks, was investigated and compared with that of equally old non-achondroplastic sibs. Differences in body weight and dry weight of femora, tibiae, humeri, and unlae, and in the length of tibiae, unlae, and humeri became more marked with advancing age. Of the biochemical assays carried out, only those for sialic acid and hydroxyproline disclosed significant differences between dwarfs and non-dwarfs, all dwarfs having higher levels of hydroxyproline, and most of them having higher sialic acid levels than the non-dwarfs. Histologically, no distinction could be made between growth zones of non-dwarfs, of cn/+, or +/+ genotype. Among the dwarfs, two groups differing as to the severity of the skeletal abnormality could be distinguished. The differences between dwarfs were most marked in the growth zones of tibiae and femora, less conspicuous in those of vertebrae and least pronounced at the chondro-osseous junction of the ribs. The cause or causes of the histologic heterogeneity remain unknown.
对2至7周龄的软骨发育不全(cn/cn)小鼠的骨骼发育进行了研究,并与同龄的非软骨发育不全同胞进行了比较。随着年龄的增长,股骨、胫骨、肱骨和尺骨的体重和干重差异,以及胫骨、尺骨和肱骨的长度差异变得更加明显。在所进行的生化分析中,只有唾液酸和羟脯氨酸的分析揭示了侏儒小鼠和非侏儒小鼠之间的显著差异,所有侏儒小鼠的羟脯氨酸水平较高,并且大多数侏儒小鼠的唾液酸水平高于非侏儒小鼠。组织学上,无法区分非侏儒小鼠、cn/+或+/+基因型的生长区。在侏儒小鼠中,可以区分出两组骨骼异常严重程度不同的小鼠。侏儒小鼠之间的差异在胫骨和股骨的生长区最为明显,在椎骨的生长区不太明显,在肋骨的软骨-骨交界处最不明显。组织学异质性的原因尚不清楚。