2MB0

Solution structure of hnRNP G RRM in complex with the RNA 5'-AUCAAA-3'

2MB0 の概要

• エントリーDOI: 10.2210/pdb2mb0/pdb
• NMR情報: BMRB: 19382
• 分子名称: RNA-binding motif protein, X chromosome, RNA_(5'-R(*AP*UP*CP*AP*AP*A)-3') (2 entities in total)
• 機能のキーワード: hnrnp g, splicing, rrm, smn, sma, splicing-rna complex, splicing/rna
• 由来する生物種: Homo sapiens (human)
• 細胞内の位置: Nucleus: P38159
• タンパク質・核酸の鎖数: 2
• 化学式量合計: 12297.03

構造登録者

Moursy, A.,Allain, F.H.-T.,Clery, A. (登録日: 2013-07-22, 公開日: 2014-04-09, 最終更新日: 2024-05-01)

主引用文献

Moursy, A.,Allain, F.H.,Clery, A.
Characterization of the RNA recognition mode of hnRNP G extends its role in SMN2 splicing regulation.
Nucleic Acids Res., 42:6659-6672, 2014

PubMed Abstract: Regulation of SMN2 exon 7 splicing is crucial for the production of active SMN protein and the survival of Spinal Muscular Atrophy (SMA) patients. One of the most efficient activators of exon 7 inclusion is hnRNP G, which is recruited to the exon by Tra2-β1. We report that in addition to the C-terminal region of hnRNP G, the RNA Recognition Motif (RRM) and the middle part of the protein containing the Arg-Gly-Gly (RGG) box are important for this function. To better understand the mode of action of hnRNP G in this context we determined the structure of its RRM bound to an SMN2 derived RNA. The RRM interacts with a 5'-AAN-3' motif and specifically recognizes the two consecutive adenines. By testing the effect of mutations in hnRNP G RRM and in its putative binding sites on the splicing of SMN2 exon 7, we show that it specifically binds to exon 7. This interaction is required for hnRNP G splicing activity and we propose its recruitment to a polyA tract located upstream of the Tra2-β1 binding site. Finally, our data suggest that hnRNP G plays a major role in the recruitment of the Tra2-β1/hnRNP G/SRSF9 trimeric complex to SMN2 exon 7.

PubMed: 24692659
DOI: 10.1093/nar/gku244

実験手法

SOLUTION NMR