Licht Tamar, Dor-Wollman Talia, Ben-Zvi Ayal, Rothe Gadiel, Keshet Eli
J Clin Invest. 2015 Mar 2;125(3):1319-28. doi: 10.1172/JCI79401. Epub 2015 Feb 17.
Premature birth is a major risk factor for multiple brain pathologies, notably periventricular leukomalacia (PVL), which is distinguished by bilateral necrosis of neural tissue around the ventricles and a sequela of neurological disturbances. The 2 hallmarks of brain pathologies of prematurity are a restricted gestational window of vulnerability and confinement of injury to a specific cerebral region. Here, we examined the proposition that both of these features are determined by the state of blood vessel immaturity. We developed a murine genetic model that allows for inducible and reversible VEGF blockade during brain development. Using this system, we determined that cerebral vessels mature in a centrifugal, wave-like fashion that results in sequential acquisition of a functional blood-brain barrier and exit from a VEGF-dependent phase, with periventricular vessels being the last to mature. This developmental program permitted selective ablation of periventricular vessels via episodic VEGF blockade within a specific, vulnerable gestational window. Enforced collapse of ganglionic eminence vessels and resultant periventricular neural apoptosis resulted in a PVL-like phenotype that recapitulates the primary periventricular lesion, ventricular enlargement, and the secondary cortical deficit in out-migrating GABAergic inhibitory interneurons. These findings provide an animal model that reproduces the temporal and spatial specificities of PVL and indicate that damage to VEGF-dependent, immature periventricular vessels contributes to PVL development.
早产是多种脑部病变的主要风险因素,尤其是脑室周围白质软化症(PVL),其特征是脑室周围神经组织的双侧坏死以及神经功能障碍后遗症。早产脑部病变的两个标志是脆弱的妊娠期窗口受限以及损伤局限于特定脑区。在此,我们研究了这两个特征均由血管不成熟状态决定这一观点。我们构建了一种小鼠遗传模型,该模型可在大脑发育期间实现诱导性和可逆性血管内皮生长因子(VEGF)阻断。利用这个系统,我们确定脑血管以离心的、波浪状方式成熟,这导致功能性血脑屏障的顺序获得以及从VEGF依赖阶段退出,脑室周围血管是最后成熟的。这种发育程序允许通过在特定的、脆弱的妊娠期窗口内间歇性阻断VEGF来选择性地消融脑室周围血管。神经节隆起血管的强制塌陷以及由此导致的脑室周围神经细胞凋亡产生了类似PVL的表型,重现了原发性脑室周围病变、脑室扩大以及外迁的γ-氨基丁酸(GABA)能抑制性中间神经元的继发性皮质缺陷。这些发现提供了一种重现PVL时间和空间特异性的动物模型,并表明对VEGF依赖的、不成熟的脑室周围血管的损伤促成了PVL的发展。