Center for Advanced Pain Studies, School of Behavioral and Brain Sciences, University of Texas at Dallas, Richardson, Texas, USA.
Department of Endodontics, UT Health San Antonio, San Antonio, Texas, USA.
J Comp Neurol. 2023 May;531(7):814-835. doi: 10.1002/cne.25463. Epub 2023 Feb 20.
Fragile X Mental Retardation Protein (FMRP) regulates activity-dependent RNA localization and local translation to modulate synaptic plasticity throughout the central nervous system. Mutations in the FMR1 gene that hinder or ablate FMRP function cause Fragile X Syndrome (FXS), a disorder associated with sensory processing dysfunction. FXS premutations are associated with increased FMRP expression and neurological impairments including sex dimorphic presentations of chronic pain. In mice, FMRP ablation causes dysregulated dorsal root ganglion (DRG) neuron excitability and synaptic vesicle exocytosis, spinal circuit activity, and decreased translation-dependent nociceptive sensitization. Activity-dependent, local translation is a key mechanism for enhancing primary nociceptor excitability that promotes pain in animals and humans. These works indicate that FMRP likely regulates nociception and pain at the level of the primary nociceptor or spinal cord. Therefore, we sought to better understand FMRP expression in the human DRG and spinal cord using immunostaining in organ donor tissues. We find that FMRP is highly expressed in DRG and spinal neuron subsets with substantia gelatinosa exhibiting the most abundant immunoreactivity in spinal synaptic fields. Here, it is expressed in nociceptor axons. FMRP puncta colocalized with Nav1.7 and TRPV1 receptor signals suggesting a pool of axoplasmic FMRP localizes to plasma membrane-associated loci in these branches. Interestingly, FMRP puncta exhibited notable colocalization with calcitonin gene-related peptide (CGRP) immunoreactivity selectively in female spinal cord. Our results support a regulatory role for FMRP in human nociceptor axons of the dorsal horn and implicate it in the sex dimorphic actions of CGRP signaling in nociceptive sensitization and chronic pain.
脆性 X 智力低下蛋白(FMRP)调节活性依赖的 RNA 定位和局部翻译,以调节整个中枢神经系统的突触可塑性。FMR1 基因的突变会阻碍或消除 FMRP 功能,导致脆性 X 综合征(FXS),这是一种与感觉处理功能障碍有关的疾病。FXS 前突变与 FMRP 表达增加和神经损伤有关,包括慢性疼痛的性别二态表现。在小鼠中,FMRP 缺失导致背根神经节(DRG)神经元兴奋性和突触小泡胞吐作用、脊髓回路活动以及依赖翻译的伤害性感受敏化作用的下调。活性依赖的局部翻译是增强初级伤害感受器兴奋性的关键机制,可促进动物和人类的疼痛。这些研究表明,FMRP 可能在初级伤害感受器或脊髓水平调节伤害感受和疼痛。因此,我们试图通过对器官捐献组织进行免疫染色,更好地了解人类 DRG 和脊髓中的 FMRP 表达。我们发现,FMRP 在 DRG 和脊髓神经元亚群中高度表达,其中胶状质显示出最强的脊髓突触场免疫反应性。在这里,它在伤害感受器轴突中表达。FMRP 斑点与 Nav1.7 和 TRPV1 受体信号共定位,表明轴浆中的 FMRP 池定位于这些分支的质膜相关位置。有趣的是,FMRP 斑点在女性脊髓中与降钙素基因相关肽(CGRP)免疫反应性选择性地表现出显著的共定位。我们的结果支持 FMRP 在人类背角伤害感受器轴突中的调节作用,并暗示它参与了 CGRP 信号在伤害感受敏化和慢性疼痛中的性别二态作用。