Department of Neurology, Pediatric Research Institute, Louisville, KY 40202, USA.
University of Louisville Autism Center, Louisville, KY 40217, USA.
Cells. 2022 Jul 15;11(14):2211. doi: 10.3390/cells11142211.
Autism Spectrum Disorder (ASD) is a common pediatric neurobiological disorder with up to 80% of genetic etiologies. Systems biology approaches may make it possible to test novel therapeutic strategies targeting molecular pathways to alleviate ASD symptoms. A clinical database of autism subjects was queried for individuals with a copy number variation (CNV) on microarray, Vineland, and Parent Concern Questionnaire scores. Pathway analyses of genes from pathogenic CNVs yielded 659 genes whose protein-protein interactions and mRNA expression mapped 121 genes with maximal antenatal expression in 12 brain regions. A Research Domain Criteria (RDoC)-derived neural circuits map revealed significant differences in anxiety, motor, and activities of daily living skills scores between altered CNV genes and normal microarrays subjects, involving Positive Valence (reward), Cognition (IQ), and Social Processes. Vascular signaling was identified as a biological process that may influence these neural circuits. Neuroinflammation, microglial activation, iNOS and 3-nitrotyrosine increase in the brain of Semaphorin 3F- Neuropilin 2 (Sema 3F-NRP2) KO, an ASD mouse model, agree with previous reports in the brain of ASD individuals. Signs of platelet deposition, activation, release of serotonin, and albumin leakage in ASD-relevant brain regions suggest possible blood brain barrier (BBB) deficits. Disruption of neurovascular signaling and BBB with neuroinflammation may mediate causative pathophysiology in some ASD subgroups. Although preliminary, these data demonstrate the potential for developing novel therapeutic strategies based on clinically derived data, genomics, cognitive neuroscience, and basic neuroscience methods.
自闭症谱系障碍 (ASD) 是一种常见的儿科神经生物学障碍,其遗传病因高达 80%。系统生物学方法可能使我们能够测试针对分子途径的新型治疗策略,以减轻 ASD 症状。对自闭症患者的临床数据库进行了微阵列、Vineland 和父母关注问卷评分的拷贝数变异 (CNV) 个体查询。致病性 CNV 基因的途径分析产生了 659 个基因,这些基因的蛋白质-蛋白质相互作用和 mRNA 表达映射了 121 个在 12 个大脑区域具有最大产前表达的基因。基于研究领域标准 (RDoC) 的神经回路图谱显示,改变的 CNV 基因与正常微阵列个体之间的焦虑、运动和日常生活技能评分存在显著差异,涉及正性效价(奖励)、认知(智商)和社会过程。血管信号被确定为可能影响这些神经回路的生物学过程。在 Semaphorin 3F-Neuropilin 2 (Sema 3F-NRP2) KO 自闭症小鼠模型的大脑中,发现了神经炎症、小胶质细胞激活、iNOS 和 3-硝基酪氨酸增加,这与 ASD 个体大脑中的先前报道一致。血小板沉积、激活、血清素释放和 ASD 相关脑区白蛋白渗漏的迹象表明可能存在血脑屏障 (BBB) 缺陷。神经血管信号和 BBB 的破坏与神经炎症可能在某些 ASD 亚组中介导因果病理生理学。尽管初步,但这些数据表明,基于临床衍生数据、基因组学、认知神经科学和基础神经科学方法,开发新型治疗策略具有潜力。