Buhusi Mona, Schlatter Monika C, Demyanenko Galina P, Thresher Randy, Maness Patricia F
Department of Biochemistry, University of North Carolina School of Medicine, Chapel Hill, North Carolina 27599, USA.
J Neurosci. 2008 Jan 2;28(1):177-88. doi: 10.1523/JNEUROSCI.3573-07.2008.
Dynamic modulation of adhesion provided by anchorage of axonal receptors with the cytoskeleton contributes to attractant or repellent responses that guide axons to topographic targets in the brain. The neural cell adhesion molecule L1 engages the spectrin-actin cytoskeleton through reversible linkage of its cytoplasmic domain to ankyrin. To investigate a role for L1 association with the cytoskeleton in topographic guidance of retinal axons to the superior colliculus, a novel mouse strain was generated by genetic knock-in that expresses an L1 point mutation (Tyr1229His) abolishing ankyrin binding. Axon tracing revealed a striking mistargeting of mutant ganglion cell axons from the ventral retina, which express high levels of ephrinB receptors, to abnormally lateral sites in the contralateral superior colliculus, where they formed multiple ectopic arborizations. These axons were compromised in extending interstitial branches in the medial direction, a normal response to the high medial to low lateral SC gradient of ephrinB1. Furthermore, ventral but not dorsal L1(Y1229H) retinal cells were impaired for ephrinB1-stimulated adhesion through beta1 integrins in culture. The retinocollicular phenotype of the L1(Tyr1229His) mutant provides the first evidence that L1 regulates topographic mapping of retinal axons through adhesion mediated by linkage to the actin cytoskeleton and functional interaction with the ephrinB/EphB targeting system.
轴突受体与细胞骨架的锚定所提供的黏附动态调节,有助于产生吸引或排斥反应,引导轴突到达大脑中的拓扑靶点。神经细胞黏附分子L1通过其胞质结构域与锚蛋白的可逆连接,与血影蛋白-肌动蛋白细胞骨架结合。为了研究L1与细胞骨架的结合在视网膜轴突向中脑上丘的拓扑导向中的作用,通过基因敲入产生了一种新型小鼠品系,该品系表达一种消除锚蛋白结合的L1点突变(Tyr1229His)。轴突追踪显示,来自腹侧视网膜的突变神经节细胞轴突出现明显的靶向错误,腹侧视网膜表达高水平的ephrinB受体,这些轴突错误地投射到对侧中脑上丘的异常外侧部位,并在那里形成多个异位分支。这些轴突在内侧方向延伸间质分支的能力受损,而这是对ephrinB1从内侧高到外侧低的正常中脑上丘梯度的正常反应。此外,在培养中,腹侧而非背侧的L1(Y1229H)视网膜细胞通过β1整合素对ephrinB1刺激的黏附受损。L1(Tyr1229His)突变体的视网膜-中脑上丘表型提供了首个证据,表明L1通过与肌动蛋白细胞骨架的连接介导的黏附以及与ephrinB/EphB靶向系统的功能相互作用,调节视网膜轴突的拓扑图谱。