Abnormal Distribution of Glycosaminoglycans in the Mandibular Condyle of Osteopetrotic (op/op) Mice

概要

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Osteopetrosis is a metabolic bone disease characterized by generalized skeletal sclerosis. In mandibular condyle of the op/op mice, one of the osteopetrotic models, deformation of the condylar head and enlargement of the hypertrophic zone are known to occur. The objective of the present study was to characterize the distribution of glycosaminoglycans (GAGs) in the mandibular condyle of op/op mice as a function of age (2, 4, 8 and 16 weeks of age) using histochemical and immunohistochemical methods. At 8 weeks of age and older, a significantly enlarged hypertrophic zone in the central condylar head of op/op mice was intensely and diffusely stained for chondroitin-4-sulfate, chondroitin-6-sulfate and hyaluronic acid, while these GAGs appeared to be present essentially in the condylar head, i.e., surface to sub-perichondrium, in the control of the same age group. In both groups, cells in sub-perichondrium, blood vessels and periosteum showed immunoreactivity for keratan sulfate. In the mineralizing matrix zone in the condyle, op/op mice showed nonuniform patches of staining for these GAG components (except for keratan sulfate) while the control showed virtually no staining. The abnormal metabolism and distribution of GAGs/proteoglycans may in part contribute to the deformation of the condylar head and its poor mineralization seen in these op/op mutant mice.
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