Article
The Alazami Syndrome-Associated Protein LARP7 Guides U6 Small Nuclear RNA Modification and Contributes to Splicing Robustness.
Molecular cell - 5 Mar 2020
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
Abstract excerpt
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 box C/D small...
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