van Tran P, Dryll A, Lansaman J, Naveau B, Treve R, Miravet L, Ryckewaert A
Virchows Arch A Pathol Anat Histopathol. 1985;408(2-3):269-80. doi: 10.1007/BF00707989.
In order to investigate skeletal abnormalities in a case of idiopathic osteopetrosis, a bone biopsy was taken from the anterior iliac crest and prepared for ultrastructural and histochemical study. There was a drastic reduction in osteoclastic bone resorption. The ruffle border and sealing zone, which are the osteoclast cell surface markers of bone resorption, were absent. The cells were highly vacuolated, and the vacuoles contained large amounts of a residual organic material which reacted strongly with acid phosphatase. Acid phosphatase activity was never found outside the cell, and in particular, not at the bone-cell interface. This suggests that the defect in bone resorption is caused by cell membrane abnormalities and the lack of ruffle border formation, rather than the inability of the lysosomal enzymes to digest the bone matrix.
为研究一例特发性骨质石化症患者的骨骼异常情况，从前髂嵴处取骨活检样本，准备用于超微结构和组织化学研究。破骨细胞介导的骨吸收显著减少。骨吸收的破骨细胞表面标志物——皱褶缘和封闭区缺失。细胞高度空泡化，空泡内含有大量与酸性磷酸酶反应强烈的残余有机物质。酸性磷酸酶活性从未在细胞外发现，尤其是在骨细胞界面处未发现。这表明骨吸收缺陷是由细胞膜异常和缺乏皱褶缘形成所致，而非溶酶体酶无法消化骨基质。
