Discovery, InsideOutBio, 42, 8th Street, Unit 3412, Charlestown, MA 02129 USA.
Commun Biol. 2019 Jan 7;2:7. doi: 10.1038/s42003-018-0237-x. eCollection 2019.
Left-handed Z-DNA/Z-RNA is bound with high affinity by the Zα domain protein family that includes ADAR (a double-stranded RNA editing enzyme), ZBP1 and viral orthologs regulating innate immunity. Loss-of-function mutations in ADAR p150 allow persistent activation of the interferon system by Alu dsRNAs and are causal for Aicardi-Goutières Syndrome. Heterodimers of ADAR and DICER1 regulate the switch from RNA- to protein-centric immunity. Loss of DICER1 function produces age-related macular degeneration, a different type of Alu-mediated disease. The overlap of Z-forming sites with those for the signal recognition particle likely limits invasion of primate genomes by Alu retrotransposons.
左手 Z-DNA/Z-RNA 与 Zα 结构域蛋白家族具有高亲和力结合，该家族包括 ADAR（双链 RNA 编辑酶）、ZBP1 和调节先天免疫的病毒同源物。ADAR p150 的功能丧失突变允许 Alu dsRNA 持续激活干扰素系统，是 Aicardi-Goutières 综合征的原因。ADAR 和 DICER1 的异二聚体调节从 RNA 到蛋白中心免疫的转换。DICER1 功能丧失会导致年龄相关性黄斑变性，这是一种不同类型的 Alu 介导疾病。信号识别颗粒的形成位点与 Z 形成位点的重叠可能限制了 Alu 反转录转座子对灵长类基因组的入侵。
