Key result
Achondroplastic (cn/cn) mice exhibited retarded longitudinal bone growth due to a significantly reduced hypertrophic zone of the growth cartilage compared to normal mice.
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Reduced hypertrophic zone drives growth failure in this model; leaves open human translation and therapeutic targeting.
The autosomal recessive gene for achondroplasia (cn) in the homozygous condition inhibits the growth of the cartilaginous skeleton in mice. Changes in the width of the proximal growth plate of the tibia and the caudal vertebrae and in the number of proliferative and hypertrophic cells were recorded in normal and achondroplastic (cn/cn) mice during the first 30 neonatal days. In normal mice as in achondroplastics, the width of the cartilaginous growth plate decreased progressively with increasing age. The proliferative zone of cn/cn mice was similar in structure to that of normal mice and showed no significant difference. Hypertrophied cartilage was less wide and there were fewer hypertrophic cells at the epiphyseal plates than found in normal mice. The data of the present study show that retarded longitudinal bone growth in cn/cn mice is due to reduced hypertrophy of the chondrocytes.
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Agustina Marco (1981) studied Achondroplasia (n=48). Achondroplasia (cn/cn mutation) vs. Normal mice (cn/+ or +/+) was evaluated on Increase in the width of the hypertrophic zone between neonatal days 1 and 5. Achondroplastic (cn/cn) mice exhibited retarded longitudinal bone growth due to a significantly reduced hypertrophic zone of the growth cartilage compared to normal mice.
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