Forino Nicholas M, Woo Jia Zheng, Zaug Arthur J, Jimenez Arcelia Gonzalez, Edelson Eva, Cech Thomas R, Rouskin Silvi, Stone Michael D
Department of Molecular, Cell, and Developmental Biology, University of California, Santa Cruz, CA, USA.
Department of Microbiology, Harvard Medical School, Boston, MA, USA.
Nat Commun. 2025 Jan 22;16(1):925. doi: 10.1038/s41467-025-56149-6.
Biogenesis of human telomerase requires its RNA subunit (hTR) to fold into a multi-domain architecture that includes the template-pseudoknot (t/PK) and the three-way junction (CR4/5). These hTR domains bind the telomerase reverse transcriptase (hTERT) protein and are essential for telomerase activity. Here, we probe hTR structure in living cells using dimethyl sulfate mutational profiling with sequencing (DMS-MaPseq) and ensemble deconvolution analysis. Approximately 15% of the steady state population of hTR has a CR4/5 conformation lacking features required for hTERT binding. The proportion of hTR CR4/5 folded into the primary functional conformation is independent of hTERT expression levels. Mutations that stabilize the alternative CR4/5 conformation are detrimental to telomerase assembly and activity. Moreover, the alternative CR4/5 conformation is not found in purified telomerase RNP complexes, supporting the hypothesis that only the primary CR4/5 conformer is active. We propose that this misfolded portion of the cellular hTR pool is either slowly refolded or degraded, suggesting that kinetic RNA folding traps studied in vitro may also hinder ribonucleoprotein assembly in vivo.
人类端粒酶的生物发生需要其RNA亚基(hTR)折叠成一种多结构域架构,该架构包括模板假结(t/PK)和三向接头(CR4/5)。这些hTR结构域结合端粒酶逆转录酶(hTERT)蛋白,对端粒酶活性至关重要。在这里,我们使用硫酸二甲酯突变谱测序(DMS-MaPseq)和整体去卷积分析来探测活细胞中的hTR结构。大约15%的hTR稳态群体具有缺乏hTERT结合所需特征的CR4/5构象。折叠成主要功能构象的hTR CR4/5比例与hTERT表达水平无关。稳定替代CR4/5构象的突变对端粒酶组装和活性有害。此外,在纯化的端粒酶RNP复合物中未发现替代CR4/5构象,这支持了只有主要的CR4/5构象体具有活性的假设。我们提出,细胞hTR池的这一错误折叠部分要么缓慢重新折叠,要么降解,这表明体外研究的动力学RNA折叠陷阱也可能阻碍体内核糖核蛋白的组装。