Dickson G R, Mollan R A, Carr K E
Department of Anatomy, Queen's University of Belfast, Northern Ireland.
Histochemistry. 1987;87(6):569-72. doi: 10.1007/BF00492472.
This report is the first cytochemical investigation of vanishing bone disease "Gorham's Disease" (Gorham and Stout 1955). The ultrastructural localization of non-specific alkaline phosphatase and of specific and non-specific acid phosphatase activity was studied in slices of tissue removed from a patient with this rare disorder. Sodium beta-glycerophosphate and phosphorylcholine chloride were used as substrates. Alkaline phosphatase was present around the plasma membranes of osteoblasts and associated with extracellular matrix vesicles in new woven bone. This is consistent with the proposed role for this enzyme (Robison 1923) and for matrix vesicles (Bonucci 1967) in the mineralization of bone (Bernard and Marvaso 1981). Concentrations of specific secretory acid phosphatase reaction product in the cytoplasm of degenerating osteoblasts may contribute to the imbalance between bone formation and resorption. Osteoclasts, while few in number, showed non-specific and specific acid phosphatase activity. The Golgi apparatus and heterophagic lysosomes of mononuclear phagocytes were rich in non-specific acid phosphatase. This was also present in the Golgi lamellae and lysosomes of endothelial cells. Acid phosphatase cytochemistry suggests that mononuclear phagocytes, multinuclear osteoclasts and the vascular endothelium are involved in bone resorption in this disease.
本报告是关于“消失性骨病——戈勒姆病”（戈勒姆和斯托特，1955年）的首次细胞化学研究。对一名患有这种罕见疾病的患者切除的组织切片中，非特异性碱性磷酸酶以及特异性和非特异性酸性磷酸酶活性的超微结构定位进行了研究。使用β-甘油磷酸钠和氯化磷酸胆碱作为底物。碱性磷酸酶存在于成骨细胞的质膜周围，并与新编织骨中的细胞外基质小泡相关。这与该酶（罗宾逊，1923年）和基质小泡（博努奇，1967年）在骨矿化中的作用（伯纳德和马尔瓦索，1981年）一致。退化的成骨细胞质中特异性分泌酸性磷酸酶反应产物的浓度可能导致骨形成与吸收之间的失衡。破骨细胞数量虽少，但显示出非特异性和特异性酸性磷酸酶活性。单核吞噬细胞的高尔基体和异噬性溶酶体富含非特异性酸性磷酸酶。这在内皮细胞的高尔基体片层和溶酶体中也有存在。酸性磷酸酶细胞化学表明，单核吞噬细胞、多核破骨细胞和血管内皮参与了该疾病中的骨吸收过程。
