1d3b

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spinqualitylabelsall models 1d3b, resolution 2.00Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image

CRYSTAL STRUCTURE OF THE D3B SUBCOMPLEX OF THE HUMAN CORE SNRNP DOMAIN AT 2.0A RESOLUTION

Contents 1 Overview 2 Disease 3 About this Structure 4 Reference

Overview

The U1, U2, U4/U6, and U5 small nuclear ribonucleoprotein particles (snRNPs) involved in pre-mRNA splicing contain seven Sm proteins (B/B', D1, D2, D3, E, F, and G) in common, which assemble around the Sm site present in four of the major spliceosomal small nuclear RNAs (snRNAs). These proteins share a common sequence motif in two segments, Sm1 and Sm2, separated by a short variable linker. Crystal structures of two Sm protein complexes, D3B and D1D2, show that these proteins have a common fold containing an N-terminal helix followed by a strongly bent five-stranded antiparallel beta sheet, and the D1D2 and D3B dimers superpose closely in their core regions, including the dimer interfaces. The crystal structures suggest that the seven Sm proteins could form a closed ring and the snRNAs may be bound in the positively charged central hole.

Disease

Known diseases associated with this structure: Cone dystrophy-1 OMIM:[312610], Cone-rod dystrophy OMIM:[312610], Macular degeneration, X-linked atrophic OMIM:[312610], Retinitis pigmentosa, X-linked with deafness and sinorespiratory infections OMIM:[312610], Retinitis pigmentosa, X-linked, with recurrent respiratory infections OMIM:[312610], Retinitis pigmentosa-3 OMIM:[312610]

About this Structure

1D3B is a Protein complex structure of sequences from Homo sapiens with and as ligands. Full crystallographic information is available from OCA.

Reference

Crystal structures of two Sm protein complexes and their implications for the assembly of the spliceosomal snRNPs., Kambach C, Walke S, Young R, Avis JM, de la Fortelle E, Raker VA, Luhrmann R, Li J, Nagai K, Cell. 1999 Feb 5;96(3):375-87. PMID:10025403

Page seeded by OCA on Thu Feb 21 12:12:29 2008