Homeostatic Plasticity in Mouse Model of Jordan's Syndrome

摘要 导致自闭症和与严重智力障碍相关的综合征的基因突变的鉴定 残疾极大地推动了研究和新的治疗方法的发展。然而， 几乎在每一种情况下，对基因功能丧失到受损的脑功能仍然知之甚少。有一个 迫切需要阐明个别基因突变如何导致大脑功能缺陷，并有可能 确定治疗干预的共同基础，使最大的患者群体受益。最近， 稳态可塑性被证明具有抵抗神经疾病发作的有效能力， 进展这被称为稳态神经保护。根据初步数据，我们建议， 稳态神经保护的机制也适用于受损神经元的发病和进展。 海马功能，基于对一种新的严重智力残疾小鼠模型的研究。这项工作 有可能为精神疾病的治疗开发开辟一条新途径。

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.