Histological and histochemical investigations of achondroplastic mice: a possible model of human achondroplasia.
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.
D000130:Achondroplasia / Q000378:metabolism / Q000473:pathology*; D000367:Age Factors; D000818:Animals; D001842:Bone and Bones / Q000378:metabolism; D002356:Cartilage / Q000378:metabolism; D003094:Collagen / Q000378:metabolism; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D011509:Proteoglycans / Q000378:metabolism
D011509:Proteoglycans; D003094:Collagen