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卷曲蛋白 4 对于正常的骨骼获得是必需的,尽管卷曲蛋白 8 可以代偿。

Frizzled-4 is required for normal bone acquisition despite compensation by Frizzled-8.

机构信息

Department of Orthopaedic Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Program for Skeletal Disease and Tumor Microenvironment and Center for Cancer and Cell Biology, Van Andel Research Institute, Grand Rapids, Michigan.

出版信息

J Cell Physiol. 2020 Oct;235(10):6673-6683. doi: 10.1002/jcp.29563. Epub 2020 Jan 27.

Abstract

The activation of the Wnt/β-catenin signaling pathway is critical for skeletal development but surprisingly little is known about the requirements for the specific frizzled (Fzd) receptors that recognize Wnt ligands. To define the contributions of individual Fzd proteins to osteoblast function, we profiled the expression of all 10 mammalian receptors during calvarial osteoblast differentiation. Expression of Fzd4 was highly upregulated during in vitro differentiation and therefore targeted for further study. Mice lacking Fzd4 in mature osteoblasts had normal cortical bone structure but reduced cortical tissue mineral density and also exhibited an impairment in the femoral trabecular bone acquisition that was secondary to a defect in the mineralization process. Consistent with this observation, matrix mineralization, markers of osteoblastic differentiation, and the ability of Wnt3a to stimulate the accumulation of β-catenin were reduced in cultures of calvarial osteoblasts deficient for Fzd4. Interestingly, Fzd4-deficient osteoblasts exhibited an increase in the expression of Fzd8 both in vitro and in vivo, which suggests that the two receptors may exhibit overlapping functions. Indeed, ablating a single Fzd8 allele in osteoblast-specific Fzd4 mutants produced a more severe effect on bone acquisition. Taken together, our data indicate that Fzd4 is required for normal bone development and mineralization despite compensation from Fzd8.

摘要

Wnt/β-catenin 信号通路的激活对于骨骼发育至关重要,但令人惊讶的是,对于识别 Wnt 配体的特定卷曲(Fzd)受体的要求知之甚少。为了确定特定 Fzd 蛋白对成骨细胞功能的贡献,我们在颅骨成骨细胞分化过程中对所有 10 种哺乳动物受体的表达进行了分析。Fzd4 的表达在体外分化过程中高度上调,因此成为进一步研究的目标。成熟成骨细胞中缺乏 Fzd4 的小鼠具有正常的皮质骨结构,但皮质组织矿物质密度降低,并且还表现出股骨小梁骨获取的损害,这是矿化过程缺陷的继发结果。与这一观察结果一致,颅骨成骨细胞中 Fzd4 缺乏的基质矿化、成骨细胞分化标志物以及 Wnt3a 刺激 β-catenin 积累的能力降低。有趣的是,Fzd4 缺乏的成骨细胞在体外和体内均表现出 Fzd8 表达增加,这表明这两个受体可能具有重叠的功能。事实上,在成骨细胞特异性 Fzd4 突变体中消除单个 Fzd8 等位基因会对骨骼获取产生更严重的影响。总之,我们的数据表明,尽管 Fzd8 有代偿作用,但 Fzd4 对于正常的骨骼发育和矿化是必需的。

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