2LEH

Solution structure of the core SMN-Gemin2 complex

2LEH の概要

• エントリーDOI: 10.2210/pdb2leh/pdb
• NMR情報: BMRB: 17711
• 分子名称: Survival of motor neuron protein-interacting protein 1, Survival motor neuron protein (2 entities in total)
• 機能のキーワード: spinal muscular atrophy, snrnp assembly, protein binding
• 由来する生物種: Homo sapiens (human); 詳細
• 細胞内の位置: Nucleus, gem: O14893; Cytoplasm: Q16637
• タンパク質・核酸の鎖数: 2
• 化学式量合計: 24472.51

構造登録者

Sarachan, K.L.,Valentine, K.,Gupta, K.,Moorman, V.,Gledhill, J.,Bernens, M.,Tommos, C.,Wand, A.J.,Van Duyne, G. (登録日: 2011-06-15, 公開日: 2012-06-27, 最終更新日: 2024-05-15)

主引用文献

Sarachan, K.L.,Valentine, K.G.,Gupta, K.,Moorman, V.R.,Gledhill Jr, J.M.,Bernens, M.,Tommos, C.,Wand, A.J.,Van Duyne, G.D.
Solution structure of the core SMN-Gemin2 complex.
Biochem.J., 445:361-370, 2012

PubMed Abstract: In humans, assembly of spliceosomal snRNPs (small nuclear ribonucleoproteins) begins in the cytoplasm where the multi-protein SMN (survival of motor neuron) complex mediates the formation of a seven-membered ring of Sm proteins on to a conserved site of the snRNA (small nuclear RNA). The SMN complex contains the SMN protein Gemin2 and several additional Gemins that participate in snRNP biosynthesis. SMN was first identified as the product of a gene found to be deleted or mutated in patients with the neurodegenerative disease SMA (spinal muscular atrophy), the leading genetic cause of infant mortality. In the present study, we report the solution structure of Gemin2 bound to the Gemin2-binding domain of SMN determined by NMR spectroscopy. This complex reveals the structure of Gemin2, how Gemin2 binds to SMN and the roles of conserved SMN residues near the binding interface. Surprisingly, several conserved SMN residues, including the sites of two SMA patient mutations, are not required for binding to Gemin2. Instead, they form a conserved SMN/Gemin2 surface that may be functionally important for snRNP assembly. The SMN-Gemin2 structure explains how Gemin2 is stabilized by SMN and establishes a framework for structure-function studies to investigate snRNP biogenesis as well as biological processes involving Gemin2 that do not involve snRNP assembly.

PubMed: 22607171
DOI: 10.1042/BJ20120241

実験手法

SOLUTION NMR