Nagy M Aurel, Price Spencer, Wang Kristina, Gill Stanley P, Ren Erika, McElrath Lorna, Pajak Victoria, Deighan Sarah, Liu Bin, Liu Xiaodong, Diallo Aissatou, Lo Shih-Ching, Kleiman Robin, Henderson Christopher, Suh Junghae, Griffith Eric C, Greenberg Michael E, Hrvatin Sinisa
bioRxiv. 2024 Sep 3:2024.09.02.610875. doi: 10.1101/2024.09.02.610875.
Spinal motor neuron (MN) dysfunction is the cause of a number of clinically significant movement disorders. Despite the recent approval of gene therapeutics targeting these MN-related disorders, there are no viral delivery mechanisms that achieve MN-restricted transgene expression. In this study, chromatin accessibility profiling of genetically defined mouse MNs was used to identify candidate cis-regulatory elements (CREs) capable of driving MN-selective gene expression. Subsequent testing of these candidates identified two CREs that confer MN-selective gene expression in the spinal cord as well as reduced off-target expression in dorsal root ganglia. Within one of these candidate elements, we identified a compact core transcription factor (TF)-binding region that drives MN-selective gene expression. Finally, we demonstrate that selective spinal cord expression of this mouse CRE is preserved in non-human primates. These findings suggest that the generation of cell-type-selective viral reagents, in which cell-type-selective CREs drive restricted gene expression, will be valuable research tools in mice and other mammalian species, with potentially significant therapeutic value in humans.
脊髓运动神经元(MN)功能障碍是许多具有临床意义的运动障碍的病因。尽管最近批准了针对这些与MN相关疾病的基因疗法,但尚无能够实现MN限制性转基因表达的病毒递送机制。在本研究中,利用基因定义的小鼠MN的染色质可及性分析来鉴定能够驱动MN选择性基因表达的候选顺式调控元件(CRE)。对这些候选元件的后续测试确定了两个CRE,它们在脊髓中赋予MN选择性基因表达,并降低了背根神经节中的脱靶表达。在这些候选元件之一中,我们鉴定出一个紧凑的核心转录因子(TF)结合区域,该区域驱动MN选择性基因表达。最后,我们证明了这种小鼠CRE在非人类灵长类动物中保留了选择性脊髓表达。这些发现表明,由细胞类型选择性CRE驱动限制性基因表达的细胞类型选择性病毒试剂的产生,将成为小鼠和其他哺乳动物物种中有价值的研究工具,对人类可能具有重大治疗价值。