skeleton
• exhibit abnormal type II collagen fibrillogenesis and fibril organization in the matrix of the zone of maturation as early as E15 and particularly at P1
• fibrils are bundled in a single direction and show an increase in diameter
• however, do not show disorganization of the chondrocytes of the growth plate, deposition of matrix molecules such as aggrecan or type X collagen, or abnormal skeletal formation
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