Perez Yonatan, Velmeshev Dmitry, Wang Li, White Matthew L, Siebert Clara, Baltazar Jennifer, Zuo Guolong, Moriano Juan Andrés, Chen Songcang, Steffen David M, Dutton Natalia Garcia, Wang Shaohui, Wick Brittney, Haeussler Maximilian, Chamberlain Stormy, Alvarez-Buylla Arturo, Kriegstein Arnold
Eli and Edythe Broad Center of Regeneration Medicine and Stem Cell Research, University of California, San Francisco, San Francisco, CA, USA.
Department of Neurology, University of California, San Francisco, San Francisco, CA, USA.
Nat Commun. 2025 Jul 4;16(1):6177. doi: 10.1038/s41467-025-61184-4.
Duplication 15q (dup15q) syndrome is a leading genetic cause of autism spectrum disorder, offering a key model for studying autism-related mechanisms. Using single-cell and single-nucleus RNA sequencing of cortical organoids from dup15q patient-derived iPSCs and post-mortem brain samples, we identify increased glycolysis, disrupted layer-specific marker expression, and aberrant morphology in deep-layer neurons during fetal-stage organoid development. In adolescent-adult postmortem brains, upper-layer neurons exhibit heightened transcriptional burden related to synaptic signaling, a pattern shared with idiopathic autism. Using spatial transcriptomics, we confirm these cell-type-specific disruptions in brain tissue. By gene co-expression network analysis, we reveal disease-associated modules that are well preserved between postmortem and organoid samples, suggesting metabolic dysregulation that may lead to altered neuron projection, synaptic dysfunction, and neuron hyperexcitability in dup15q syndrome.
15号染色体重复(dup15q)综合征是自闭症谱系障碍的主要遗传病因,为研究自闭症相关机制提供了关键模型。通过对源自dup15q患者的诱导多能干细胞(iPSC)和死后大脑样本的皮质类器官进行单细胞和单细胞核RNA测序,我们发现在胎儿期类器官发育过程中,深层神经元的糖酵解增加、层特异性标志物表达紊乱以及形态异常。在青少年至成人的死后大脑中,上层神经元表现出与突触信号传导相关的转录负担加重,这一模式与特发性自闭症相同。利用空间转录组学,我们在脑组织中证实了这些细胞类型特异性的破坏。通过基因共表达网络分析,我们揭示了在死后样本和类器官样本之间保存良好的疾病相关模块,提示代谢失调可能导致dup15q综合征中神经元投射改变、突触功能障碍和神经元过度兴奋。