Alu elements, highly abundant short interspersed nuclear elements (SINEs) in the human genome, can form inverted Alu repeats (IRAlus) when two elements with opposite orientations are positioned close to each other. IRAlus are key immunogenic self-dsRNAs and post-transcriptional cis-regulatory elements that play roles in circular RNA biogenesis, RNA transport, and stability. They have emerged as regulators of transcription and activators of Z-DNA-binding proteins. The formation and activity of IRAlus can be modulated through RNA editing and interactions with RNA-binding proteins, and their misregulation has been implicated in immune-associated disorders. This review summarizes the emerging functions of IRAlus, including their role as immunogenic self-dsRNAs and post-transcriptional gene regulators. It highlights how IRAlus are recognized by innate immune sensors, such as MDAS and PKR, and their involvement in immune-related diseases. The review also discusses the regulatory mechanisms governing IRAlus activity, including RNA editing by ADAR1 and interactions with RBPs like TDP-43 and Dicer. Additionally, it explores the gene regulatory roles of IRAlus, particularly in alternative splicing, circular RNA biogenesis, and mRNA stability and nuclear sequestration. The review concludes by discussing future perspectives on the regulation and application of IRAlus, including their potential as biomolecular condensates, the role of m6A modification, and the possibility of using synthetic Alu RNA for cancer immunotherapy. Overall, the multifaceted nature of IRAlus and their significance in both physiological and pathological processes are emphasized.Alu elements, highly abundant short interspersed nuclear elements (SINEs) in the human genome, can form inverted Alu repeats (IRAlus) when two elements with opposite orientations are positioned close to each other. IRAlus are key immunogenic self-dsRNAs and post-transcriptional cis-regulatory elements that play roles in circular RNA biogenesis, RNA transport, and stability. They have emerged as regulators of transcription and activators of Z-DNA-binding proteins. The formation and activity of IRAlus can be modulated through RNA editing and interactions with RNA-binding proteins, and their misregulation has been implicated in immune-associated disorders. This review summarizes the emerging functions of IRAlus, including their role as immunogenic self-dsRNAs and post-transcriptional gene regulators. It highlights how IRAlus are recognized by innate immune sensors, such as MDAS and PKR, and their involvement in immune-related diseases. The review also discusses the regulatory mechanisms governing IRAlus activity, including RNA editing by ADAR1 and interactions with RBPs like TDP-43 and Dicer. Additionally, it explores the gene regulatory roles of IRAlus, particularly in alternative splicing, circular RNA biogenesis, and mRNA stability and nuclear sequestration. The review concludes by discussing future perspectives on the regulation and application of IRAlus, including their potential as biomolecular condensates, the role of m6A modification, and the possibility of using synthetic Alu RNA for cancer immunotherapy. Overall, the multifaceted nature of IRAlus and their significance in both physiological and pathological processes are emphasized.