Role of the polycytin-primary cilia complex in bone development and mechanosensing.
Role of the polycytin-primary cilia complex in bone development and mechanosensing.
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DOI：
10.1111/j.1749-6632.2009.05239.x
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发表时间：
2010-03
影响因子：
5.2
通讯作者：
Quarles LD
中科院分区：
文献类型：
--
作者：
Xiao ZS;Quarles LD
Pkd1编码PC1（一种跨膜受体样蛋白），Pkd2编码PC2（一种钙通道），两者相互作用形成功能性多囊蛋白复合物，广泛表达于许多组织和细胞类型中。由PKD1或PKD2基因失活突变引起的常染色体显性多囊肾病（ADPKD）的研究已经阐明了多囊蛋白的功能及其对肾上皮细胞初级纤毛的依赖性。我们发现Pkd1和Pkd2以及初级纤毛存在于成骨细胞和骨细胞中。此外，我们发现小鼠多囊蛋白-1 （Pkd1）功能的缺失会导致成骨细胞分化受损导致骨发育异常和骨质减少。这很可能是多细胞素/初级纤毛复合体对影响骨骼发育和骨形成的众多环境线索作出反应。总之，骨中的多胱氨酸可能为开发合成代谢药物治疗骨质疏松症提供了新的靶点。
Pkd1 encodes PC1, a transmembrane receptor-like protein, and Pkd2 encodes PC2, a calcium channel, which interact to form functional polycystin complexes that are widely expressed in many tissues and cell types. The study of autosomal dominant polycystic kidney disease (ADPKD), caused by inactivating mutations of PKD1 or PKD2 genes, has elucidated the functions of polycystins and their interdependence on primary cilia in renal epithelial cells. We have found that Pkd1 and Pkd2, as well as primary cilia, are present in osteoblasts and osteocytes. In addition, we have found that loss of polycystin-1 (Pkd1) function in mice results in abnormal bone development and osteopenia due to the impaired differentiation of osteoblasts. It is likely that the polycytin/primary cilia complex responds to a multitude of environmental clues affecting skeletal development and bone formation postnatally. Overall, polycystins in bone may define a new target for developing anabolic agents to treat osteoporotic disorders.
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影响因子：
4.1
作者：
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通讯作者：
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影响因子：
13.6
作者：
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影响因子：
4.8
作者：
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通讯作者：
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影响因子：
6
作者：
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通讯作者：
Gubler, MC
DOI：
10.1073/pnas.211191098
发表时间：
2001-10-09
影响因子：
11.1
作者：
Boulter, C;Mulroy, S;Sandford, R
通讯作者：
Sandford, R