ATYPICAL MULTINUCLEATED CELLS FORM IN LONG-TERM MARROW CULTURES FROM PATIENTS WITH PAGETS-DISEASE
ATYPICAL MULTINUCLEATED CELLS FORM IN LONG-TERM MARROW CULTURES FROM PATIENTS WITH PAGETS-DISEASE
复制标题
DOI：
10.1172/jci114565
复制
发表时间：
1990-04-01
影响因子：
15.9
通讯作者：
ROODMAN, GD
中科院分区：
文献类型：
--
作者：
KUKITA, A;CHENU, C;ROODMAN, GD
尽管佩吉特病是原发性破骨细胞紊乱的最明显的例子，但对这种疾病中的破骨细胞生物学知之甚少。在本报告中，我们研究了佩吉特病患者骨髓单核细胞长期培养中具有破骨细胞表型的细胞的形成，并将其与正常人长期骨髓培养中形成的类似细胞以及破骨细胞进行了比较。存在于pagetic骨中。在pagetic骨髓培养中形成的破骨细胞类似于pagetic骨中存在的破骨细胞，但与正常骨髓培养中形成的破骨细胞明显不同。破骨细胞的形成是正常骨髓培养的10-20倍。pagetic骨髓培养物中形成的多核细胞的大小要大得多，对1，25（OH）2维生素D反应过度，每个细胞有更多的核，抗酒石酸酸性磷酸酶活性水平增加，并具有正常骨髓单核细胞形成的多核细胞中未见的超微结构特征。这些pagetic骨髓来源的多核细胞形成大的吸收陷窝的钙化基质和交叉反应的单克隆抗体，优先结合破骨细胞。从佩吉特患者未受累部位获得的骨髓中形成的多核细胞也显示出这些异常特征。
Although Paget''s disease is the most flagrant example of a primary osteoclast disorder, little is know of osteoclast biology in this disease. In this report we have studied the formation of cells with the osteoclast phenotype in long-term cultures of marrow mononuclear cells derived from patients with Paget''s disease, and compared these with similar cells formed in long-term marrow cultures from normal individuals, and with osteoclasts present in pagetic bone. Osteoclasts formed in pagetic marrow cultures resemble osteoclasts present in pagetic bone, but were distinctly different from osteoclasts formed in normal marrow cultures. Osteoclast formation was 10-20 fold greater in pagetic marrow cultures than in normal cultures. The mutinucleated cells formed in cultures of pagetic marrow were much larger in size, were hyperresponsive to 1,25(OH)2 vitamin D, had more nuclei per cell, had increased levels of tartrate-resistant acid phosphatase activity and had ultrastructural features which were not seen in multinucleated cells formed from normal marrow mononuclear cells. These pagetic marrow-derived multinucleated cells formed large resorption lacunae on calcified matrices and cross-reacted with monoclonal antibodies which preferentially bind to osteoclasts. Tthe multinucleated cells formed from marrow obtained from uninvolved sites in Paget''s patients also displayed these abnormal features.