Department of Physiology & Biomedical Engineering, Mayo Clinic, Rochester, MN 55902, USA.
Int J Mol Sci. 2023 Apr 9;24(8):6962. doi: 10.3390/ijms24086962.
During development, GABA and glycine play major trophic and synaptic roles in the establishment of the neuromotor system. In this review, we summarise the formation, function and maturation of GABAergic and glycinergic synapses within neuromotor circuits during development. We take special care to discuss the differences in limb and respiratory neuromotor control. We then investigate the influences that GABAergic and glycinergic neurotransmission has on two major developmental neuromotor disorders: Rett syndrome and spastic cerebral palsy. We present these two syndromes in order to contrast the approaches to disease mechanism and therapy. While both conditions have motor dysfunctions at their core, one condition Rett syndrome, despite having myriad symptoms, has scientists focused on the breathing abnormalities and their alleviation-to great clinical advances. By contrast, cerebral palsy remains a scientific quagmire or poor definitions, no widely adopted model and a lack of therapeutic focus. We conclude that the sheer abundance of diversity of inhibitory neurotransmitter targets should provide hope for intractable conditions, particularly those that exhibit broad spectra of dysfunction-such as spastic cerebral palsy and Rett syndrome.
在发育过程中,GABA 和甘氨酸在运动神经系统的建立中发挥主要的营养和突触作用。在这篇综述中,我们总结了运动神经回路中 GABA 能和甘氨酸能突触在发育过程中的形成、功能和成熟。我们特别注意讨论了肢体和呼吸运动神经控制的差异。然后,我们研究了 GABA 能和甘氨酸能神经传递对两种主要发育性运动神经障碍的影响:雷特综合征和痉挛性脑瘫。我们提出这两种综合征是为了对比疾病机制和治疗方法的差异。虽然这两种疾病都以运动功能障碍为核心,但一种疾病——雷特综合征,尽管有多种症状,但科学家们专注于呼吸异常及其缓解——这取得了巨大的临床进展。相比之下,脑瘫仍然是一个科学的困境,或者是定义不明确、没有广泛采用的模型以及缺乏治疗重点。我们的结论是,抑制性神经递质靶点的多样性应该为治疗棘手疾病,特别是那些表现出广泛功能障碍的疾病,如痉挛性脑瘫和雷特综合征,提供希望。