Patterns of matrix vesicle calcification in osteomalacia of Gyro mice

Homozygous Gyro mice manifest widespread abnormalities in the inner ear and other parts of the skeleton. These disorders were attributed to a sex linked dominant gene. In the present study, tissue specimens were obtained from the inner ear and maxillary and tibial bones, and undecalcified samples were processed for light and transmission electron microscopy. Light microscopy revealed osseous changes consistent with osteomalacia. These alterations comprised broad bands of osteoid with partial or complete failure of calcification. Electron micrographs showed longitudinal calcifying fronts composed of hydroxyapatite crystals. Abundance of matrix vesicles could be demonstrated in the extracellular matrix. Many of the vesicles contained crystaline mineral. In addition, calcification alongside collagen fibers was recorded. Occasionally, apatite crystals were attached to plasma membranes. Female and hemizygous male mice showed normal patterns of calcification. The findings in the affected Gyro mice appear to be similar to previously reported changes in human osteomalacia. Further definition of the pathological process in this animal model may be valuable in defining mechanisms operative in the development of osteomalacia in man.