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The intellectual disability protein Oligophrenin-1 controls astrocyte morphology and migration.
Glia ( IF 6.2 ) Pub Date : 2020-02-19 , DOI: 10.1002/glia.23801 Laure-Elise Pillet 1, 2, 3 , Noémie Cresto 1 , Yoann Saillour 3 , Grégory Ghézali 1 , Alexis-Pierre Bemelmans 4 , Jean Livet 5 , Thierry Bienvenu 3 , Nathalie Rouach 1 , Pierre Billuart 3
Glia ( IF 6.2 ) Pub Date : 2020-02-19 , DOI: 10.1002/glia.23801 Laure-Elise Pillet 1, 2, 3 , Noémie Cresto 1 , Yoann Saillour 3 , Grégory Ghézali 1 , Alexis-Pierre Bemelmans 4 , Jean Livet 5 , Thierry Bienvenu 3 , Nathalie Rouach 1 , Pierre Billuart 3
Affiliation
Astrocytes are involved in several aspects of neuronal development and properties which are altered in intellectual disability (ID). Oligophrenin‐1 is a RhoGAP protein implicated in actin cytoskeleton regulation, and whose mutations are associated with X‐linked ID. Oligophrenin‐1 is expressed in neurons, where its functions have been widely reported at the synapse, as well as in glial cells. However, its roles in astrocytes are still largely unexplored. Using in vitro and in vivo models of oligophrenin1 disruption in astrocytes, we found that oligophrenin1 regulates at the molecular level the RhoA/ROCK/MLC2 pathway in astroglial cells. We also showed at the cellular level that oligophrenin1 modulates astrocyte morphology and migration both in vitro and in vivo, and is involved in glial scar formation. Altogether, these data suggest that oligophrenin1 deficiency alters not only neuronal but also astrocytic functions, which might contribute to the development of ID.
中文翻译:
智力障碍蛋白 Oligophrenin-1 控制星形胶质细胞的形态和迁移。
星形胶质细胞参与神经元发育和特性的多个方面,这些方面会在智力障碍 (ID) 中发生改变。Oligophrenin-1 是一种涉及肌动蛋白细胞骨架调节的 RhoGAP 蛋白,其突变与 X 连锁 ID 相关。Oligophrenin-1 在神经元中表达,其功能在突触和神经胶质细胞中得到广泛报道。然而,它在星形胶质细胞中的作用在很大程度上仍未得到探索。使用星形胶质细胞中 oligophrenin1 破坏的体外和体内模型,我们发现 oligophrenin1 在分子水平上调节星形胶质细胞中的 RhoA/ROCK/MLC2 通路。我们还在细胞水平上表明 oligophrenin1 在体外和体内调节星形胶质细胞的形态和迁移,并参与胶质瘢痕形成。共,
更新日期:2020-02-19
中文翻译:
智力障碍蛋白 Oligophrenin-1 控制星形胶质细胞的形态和迁移。
星形胶质细胞参与神经元发育和特性的多个方面,这些方面会在智力障碍 (ID) 中发生改变。Oligophrenin-1 是一种涉及肌动蛋白细胞骨架调节的 RhoGAP 蛋白,其突变与 X 连锁 ID 相关。Oligophrenin-1 在神经元中表达,其功能在突触和神经胶质细胞中得到广泛报道。然而,它在星形胶质细胞中的作用在很大程度上仍未得到探索。使用星形胶质细胞中 oligophrenin1 破坏的体外和体内模型,我们发现 oligophrenin1 在分子水平上调节星形胶质细胞中的 RhoA/ROCK/MLC2 通路。我们还在细胞水平上表明 oligophrenin1 在体外和体内调节星形胶质细胞的形态和迁移,并参与胶质瘢痕形成。共,