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In This Issue

机译：在这个问题上

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Cherubism is a genetic disorder characterized by craniofacial abnormalities that result from excessive bone resorption by activated osteoclasts. It is caused by mutations in the SH3-domainbinding protein 2 ( SH3BP2 ) gene that result in gain-of-function effects on the protein encoded by the gene, the adaptor protein 3BP2. To date, little is known about the normal function of wild-type 3BP2 in regulating bone homeostasis. To investigate this, Levaot and colleagues analyzed mice lacking 3BP2 (32443257). They found that these mice developed osteoporosis and that this was not due to increased bone resorption by osteoclasts but rather a result of reduced bone formation by osteoblasts. Further analysis revealed cell-intrinsic defects in both osteoblasts and osteoclasts in vivo and in vitro and determined that these defects were a result of 3BP2 being a pivotal adaptor protein required for Abl activation in osteoblasts and Src activation in osteoclasts. These data indicate that 3BP2 has a key role in regulating bone homeostasis because it is essential for the normal function of both osteoblasts and osteoclasts. .
机译：基路伯病是一种遗传性疾病，其特征是颅面异常，这是由于活化的破骨细胞过度吸收骨而引起的。它是由SH3结构域结合蛋白2（SH3BP2）基因的突变引起的，该突变导致对该基因编码的蛋白（衔接蛋白3BP2）产生功能获得作用。迄今为止，对野生型3BP2在调节骨稳态中的正常功能了解甚少。为了对此进行调查，Levaot及其同事分析了缺乏3BP2（32443257）的小鼠。他们发现这些小鼠发展成骨质疏松症，这不是由于破骨细胞增加了骨吸收，而是由于成骨细胞减少了骨形成的结果。进一步的分析揭示了体内和体外成骨细胞和破骨细胞的细胞固有缺陷，并确定这些缺陷是3BP2的结果，3BP2是成骨细胞中Abl激活和破骨细胞Src激活所需的关键衔接蛋白。这些数据表明3BP2在调节骨稳态中起关键作用，因为它对于成骨细胞和破骨细胞的正常功能至关重要。 。

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