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The Alazami Syndrome-Associated Protein LARP7 Guides U6 Small Nuclear RNA Modification and Contributes to Splicing Robustness

Hasler, Daniele ; Meduri, Rajyalakshmi ; Bąk, Maciej ; Lehmann, Gerhard ; Heizinger, Leonhard ; Wang, Xin ; Li, Zhi-Tong ; Sement, François M. ; Bruckmann, Astrid ; Dock-Bregeon, Anne-Catherine ; Merkl, Rainer ; Kalb, Reinhard ; Grauer, Eva ; Kunstmann, Erdmute ; Zavolan, Mihaela ; Liu, Mo-Fang ; Fischer, Utz ; Meister, Gunter ;


The La-related protein 7 (LARP7) forms a complex with the nuclear 7SK RNA to regulate RNA polymerase II transcription. It has been implicated in cancer and the Alazami syndrome, a severe developmental disorder. Here, we report a so far unknown role of this protein in RNA modification. We show that LARP7 physically connects the spliceosomal U6 small nuclear RNA (snRNA) with a distinct subset of ...


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