Center for Molecular Neurobiology Hamburg (ZMNH), University Medical Center Hamburg-Eppendorf, Falkenried 94, 20251, Hamburg, Germany.
Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, 20251, Hamburg, Germany.
Acta Neuropathol Commun. 2021 Nov 20;9(1):185. doi: 10.1186/s40478-021-01289-1.
LIN28A overexpression has been identified in malignant brain tumors called embryonal tumors with multilayered rosettes (ETMR) but its specific role during brain development remains largely unknown. Radial glia cells of the ventricular zone (VZ) are proposed as a cell of origin for ETMR. We asked whether an overexpression of LIN28A in such cells might affect brain development or result in the formation of brain tumors.Constitutive overexpression of LIN28A in hGFAP-cre::lsl-Lin28A (GL) mice led to a transient increase of proliferation in the cortical VZ at embryonic stages but no postnatal brain tumor formation. Postnatally, GL mice displayed a pyramidal cell layer dispersion of the hippocampus and altered spine and dendrite morphology, including reduced dendritic spine densities in the hippocampus and cortex. GL mice displayed hyperkinetic activity and differential quantitative MS-based proteomics revealed altered time dependent molecular functions regarding mRNA processing and spine morphogenesis. Phosphoproteomic analyses indicated a downregulation of mTOR pathway modulated proteins such as Map1b being involved in microtubule dynamics.In conclusion, we show that Lin28A overexpression transiently increases proliferation of neural precursor cells but it is not sufficient to drive brain tumors in vivo. In contrast, Lin28A impacts on protein abundancy patterns related to spine morphogenesis and phosphorylation levels of proteins involved in microtubule dynamics, resulting in decreased spine densities of neurons in the hippocampus and cortex as well as in altered behavior. Our work provides new insights into the role of LIN28A for neuronal morphogenesis and development and may reveal future targets for treatment of ETMR patients.
LIN28A 过表达已在称为具有多层玫瑰花结的胚胎性肿瘤(ETMR)的恶性脑肿瘤中被鉴定,但它在大脑发育过程中的具体作用仍知之甚少。室管膜下区(VZ)的放射状胶质细胞被认为是 ETMR 的起源细胞。我们想知道,这些细胞中 LIN28A 的过表达是否会影响大脑发育或导致脑瘤形成。
在 hGFAP-cre::lsl-Lin28A(GL)小鼠中,LIN28A 的组成性过表达导致胚胎阶段皮质 VZ 中的增殖短暂增加,但未导致出生后脑瘤形成。出生后,GL 小鼠表现出海马 CA1 区锥体层弥散和棘突和树突形态改变,包括海马和皮质中的树突棘密度降低。GL 小鼠表现出多动行为,基于 MS 的定量蛋白质组学差异揭示了与 mRNA 处理和棘突形态发生相关的时间依赖性分子功能的改变。磷酸化蛋白质组学分析表明,mTOR 途径调节蛋白如参与微管动力学的 Map1b 的下调。
总之,我们表明 Lin28A 过表达会短暂增加神经前体细胞的增殖,但不足以在体内驱动脑瘤的发生。相反,Lin28A 影响与棘突形态发生和参与微管动力学的蛋白质的磷酸化水平相关的蛋白质丰度模式,导致海马和皮质神经元的棘突密度降低以及行为改变。我们的工作为 LIN28A 对神经元形态发生和发育的作用提供了新的见解,并可能为治疗 ETMR 患者提供新的治疗靶点。