Molecular Neurobiology Laboratory, Salk Institute for Biological Studies, La Jolla, CA, USA.
Neurosciences Graduate Program, University of California, San Diego, La Jolla, CA, USA.
Nat Neurosci. 2022 Sep;25(9):1163-1178. doi: 10.1038/s41593-022-01150-1. Epub 2022 Aug 30.
Astrocytes negatively impact neuronal development in many models of neurodevelopmental disorders (NDs); however, how they do this, and if mechanisms are shared across disorders, is not known. In this study, we developed a cell culture system to ask how astrocyte protein secretion and gene expression change in three mouse models of genetic NDs (Rett, Fragile X and Down syndromes). ND astrocytes increase release of Igfbp2, a secreted inhibitor of insulin-like growth factor (IGF). IGF rescues neuronal deficits in many NDs, and we found that blocking Igfbp2 partially rescues inhibitory effects of Rett syndrome astrocytes, suggesting that increased astrocyte Igfbp2 contributes to decreased IGF signaling in NDs. We identified that increased BMP signaling is upstream of protein secretion changes, including Igfbp2, and blocking BMP signaling in Fragile X and Rett syndrome astrocytes reverses inhibitory effects on neurite outgrowth. This work provides a resource of astrocyte-secreted proteins in health and ND models and identifies novel targets for intervention in diverse NDs.
星形胶质细胞在许多神经发育障碍 (ND) 的模型中对神经元发育产生负面影响;然而,它们是如何做到这一点的,以及这些机制是否在不同的疾病中共享,目前尚不清楚。在这项研究中,我们开发了一种细胞培养系统,以研究三种遗传 ND 小鼠模型(雷特综合征、脆性 X 综合征和唐氏综合征)中星形胶质细胞的蛋白分泌和基因表达如何变化。ND 星形胶质细胞增加了胰岛素样生长因子 (IGF) 抑制剂 Igfbp2 的释放。IGF 可挽救许多 ND 中的神经元缺陷,我们发现阻断 Igfbp2 可部分挽救雷特综合征星形胶质细胞的抑制作用,表明增加的星形胶质细胞 Igfbp2 导致 ND 中 IGF 信号转导降低。我们发现,BMP 信号转导的增加是蛋白分泌变化(包括 Igfbp2)的上游,阻断脆性 X 综合征和雷特综合征星形胶质细胞中的 BMP 信号转导可逆转对神经突生长的抑制作用。这项工作提供了健康和 ND 模型中星形胶质细胞分泌蛋白的资源,并确定了针对不同 ND 进行干预的新靶标。