CNR, Institute of Neuroscience, Via Vanvitelli 32, 20129 Milan, Italy.
San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy.
Prog Neuropsychopharmacol Biol Psychiatry. 2018 Jun 8;84(Pt B):328-342. doi: 10.1016/j.pnpbp.2017.09.014. Epub 2017 Sep 19.
Neurodevelopmental disorders (NDDs) are a group of diseases whose symptoms arise during childhood or adolescence and that impact several higher cognitive functions such as learning, sociability and mood. Accruing evidence suggests that a shared pathogenic mechanism underlying these diseases is the dysfunction of glutamatergic synapses. We summarize present knowledge on autism spectrum disorders (ASD), intellectual disability (ID), Down syndrome (DS), Rett syndrome (RS) and attention-deficit hyperactivity disorder (ADHD), highlighting the involvement of glutamatergic synapses and receptors in these disorders. The most commonly shared defects involve α-amino-3-hydroxy-5-methyl- 4-isoxazole propionic acid receptors (AMPARs), N-methyl-d-aspartate receptors (NMDARs) and metabotropic glutamate receptors (mGluRs), whose functions are strongly linked to synaptic plasticity, affecting both cell-autonomous features as well as circuit formation. Moreover, the major scaffolding proteins and, thus, the general structure of the synapse are often deregulated in neurodevelopmental disorders, which is not surprising considering their crucial role in the regulation of glutamate receptor positioning and functioning. This convergence of defects supports the definition of neurodevelopmental disorders as a continuum of pathological manifestations, suggesting that glutamatergic synapses could be a therapeutic target to ameliorate patient symptomatology.
神经发育障碍(NDDs)是一组疾病,其症状在儿童或青少年时期出现,并影响学习、社交和情绪等多种高级认知功能。越来越多的证据表明,这些疾病的共同致病机制是谷氨酸能突触的功能障碍。我们总结了自闭症谱系障碍(ASD)、智力障碍(ID)、唐氏综合征(DS)、雷特综合征(RS)和注意缺陷多动障碍(ADHD)的现有知识,强调了谷氨酸能突触和受体在这些疾病中的作用。最常见的共同缺陷涉及α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体(AMPARs)、N-甲基-D-天冬氨酸受体(NMDARs)和代谢型谷氨酸受体(mGluRs),其功能与突触可塑性密切相关,影响细胞自主性特征和回路形成。此外,神经发育障碍中经常出现主要的支架蛋白,因此突触的一般结构也经常失调,考虑到它们在调节谷氨酸受体定位和功能方面的关键作用,这并不奇怪。这些缺陷的汇聚支持了将神经发育障碍定义为病理表现连续体的概念,表明谷氨酸能突触可能是改善患者症状的治疗靶点。