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Abstract：
To describe the contributions of osteocytes to the lesions in Paget\'s disease, which are characterized by locally overactive bone resorption and formation.
Osteocytes, the most abundant cells in bone, are altered in Paget\'s disease lesions, displaying increased size, decreased canalicular length, incomplete differentiation, and less sclerostin expression compared to controls in both patients and mouse models. Pagetic lesions show increased senescent osteocytes that express RANK ligand, which drives osteoclastic bone resorption. Abnormal osteoclasts in Paget\'s disease secrete abundant IGF1, which enhances osteocyte senescence, contributing to lesion formation. Recent data suggest that osteocytes contribute to lesion formation in Paget\'s disease by responding to high local IGF1 released from abnormal osteoclasts. Here we describe the characteristics of osteocytes in Paget\'s disease and their role in bone lesion formation based on recent results with mouse models and supported by patient data.
Osteocytes, the most abundant cells in bone, are altered in Paget\'s disease lesions, displaying increased size, decreased canalicular length, incomplete differentiation, and less sclerostin expression compared to controls in both patients and mouse models. Pagetic lesions show increased senescent osteocytes that express RANK ligand, which drives osteoclastic bone resorption. Abnormal osteoclasts in Paget\'s disease secrete abundant IGF1, which enhances osteocyte senescence, contributing to lesion formation. Recent data suggest that osteocytes contribute to lesion formation in Paget\'s disease by responding to high local IGF1 released from abnormal osteoclasts. Here we describe the characteristics of osteocytes in Paget\'s disease and their role in bone lesion formation based on recent results with mouse models and supported by patient data.
摘要：
目的：描述骨细胞对Paget病病变的贡献，其特征是局部过度活跃的骨吸收和形成。
结果：骨细胞，骨骼中最丰富的细胞，在佩吉特的疾病病变中发生了改变，显示增加的尺寸，小管长度减少，不完全分化,与患者和小鼠模型中的对照相比，硬化蛋白表达较少。Pagetic病变显示表达RANK配体的衰老骨细胞增加，驱动破骨细胞骨吸收。Paget病的异常破骨细胞分泌丰富的IGF1,促进骨细胞衰老,有助于病变形成。最近的数据表明，骨细胞通过响应异常破骨细胞释放的高局部IGF1，有助于Paget病的病变形成。在这里，我们描述了Paget病中骨细胞的特征及其在骨损伤形成中的作用，基于小鼠模型的最新结果并得到患者数据的支持。
结果：骨细胞，骨骼中最丰富的细胞，在佩吉特的疾病病变中发生了改变，显示增加的尺寸，小管长度减少，不完全分化,与患者和小鼠模型中的对照相比，硬化蛋白表达较少。Pagetic病变显示表达RANK配体的衰老骨细胞增加，驱动破骨细胞骨吸收。Paget病的异常破骨细胞分泌丰富的IGF1,促进骨细胞衰老,有助于病变形成。最近的数据表明，骨细胞通过响应异常破骨细胞释放的高局部IGF1，有助于Paget病的病变形成。在这里，我们描述了Paget病中骨细胞的特征及其在骨损伤形成中的作用，基于小鼠模型的最新结果并得到患者数据的支持。
