Homeostatic Plasticity in Mouse Model of Jordan's Syndrome

摘要 导致自闭症和严重智力障碍综合征的基因突变的鉴定 残疾极大地促进了研究和新的治疗开发。然而，联系在一起的机制 大脑功能受损所致的基因功能丧失几乎在每一种情况下都知之甚少。有一个 迫切需要阐明单个基因突变是如何导致大脑功能缺陷的，并有可能 确定治疗干预的共同依据，使最大的患者群体受益。最近， 体内平衡可塑性被证明具有强大的能力来抵消神经系统疾病的发病和 进步。这被称为动态平衡神经保护。根据初步数据，我们建议 动态平衡神经保护机制也适用于受损患者的发病和进展 海马体功能，基于对一种新的严重智力残疾小鼠模型的研究。这部作品 有可能为精神疾病的治疗发展开辟一条新的途径。

ABSTRACT The identification of gene mutations that cause autism and syndromes associated with severe intellectual disability has greatly advanced both research and new therapeutic development. Yet, the mechanisms that link gene loss of function to impaired brain function remain poorly understood in almost every instance. There is a pressing need to elucidate how individual gene mutations lead to deficits in brain function, with the potential to identify common grounds for therapeutic intervention that will benefit the largest patient population. Recently, homeostatic plasticity was demonstrated to have a potent ability to counteract neurological disease onset and progression. This is referred to as Homeostatic Neuroprotection. Based on preliminary data, we propose that the mechanisms of homeostatic neuroprotection also apply to the onset and progression of impaired hippocampal function, based on work examining a new mouse model of severe intellectual disability. This work has the potential to open a new approach toward therapeutic development in psychiatric disease.