Department of Developmental Biology, Faculty of Biology, University Duisburg-Essen, Essen, Germany.
J Bone Miner Res. 2013 Feb;28(2):246-59. doi: 10.1002/jbmr.1751.
During vertebrate skeletal development, osteoblasts produce a mineralized bone matrix by deposition of hydroxyapatite crystals in the extracellular matrix. Anoctamin6/Tmem16F (Ano6) belongs to a conserved family of transmembrane proteins with chloride channel properties. In addition, Ano6 has been linked to phosphatidylserine (PS) scrambling in the plasma membrane. During skeletogenesis, Ano6 mRNA is expressed in differentiating and mature osteoblasts. Deletion of Ano6 in mice results in reduced skeleton size and skeletal deformities. Molecular analysis revealed that chondrocyte and osteoblast differentiation are not disturbed. However, mutant mice display increased regions of nonmineralized, Ibsp-expressing osteoblasts in the periosteum during embryonic development and increased areas of uncalcified osteoid postnatally. In primary Ano6(-/-) osteoblasts, mineralization is delayed, indicating a cell autonomous function of Ano6. Furthermore, we demonstrate that calcium-dependent PS scrambling is impaired in osteoblasts. Our study is the first to our knowledge to reveal the requirement of Ano6 in PS scrambling in osteoblasts, supporting a function of PS exposure in the deposition of hydroxyapatite.
在脊椎动物骨骼发育过程中,成骨细胞通过在细胞外基质中沉积羟磷灰石晶体来产生矿化的骨基质。Anoctamin6/Tmem16F(Ano6)属于具有氯离子通道特性的保守跨膜蛋白家族。此外,Ano6 与质膜中的磷脂酰丝氨酸(PS)重排有关。在成骨过程中,Ano6 mRNA 在分化和成熟的成骨细胞中表达。在小鼠中敲除 Ano6 会导致骨骼大小减小和骨骼畸形。分子分析显示,软骨细胞和成骨细胞分化没有受到干扰。然而,突变小鼠在胚胎发育过程中骨膜中表现出未矿化的 Ibsp 表达成骨细胞的区域增加,并且出生后未钙化的类骨质区域增加。在原代 Ano6(-/-)成骨细胞中,矿化延迟,表明 Ano6 具有细胞自主功能。此外,我们证明钙依赖性 PS 重排在成骨细胞中受损。我们的研究首次揭示了 Ano6 在成骨细胞中 PS 重排中的必需性,支持 PS 暴露在羟磷灰石沉积中的功能。