2v7f

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2v7f, resolution 1.15Å

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STRUCTURE OF P. ABYSSI RPS19 PROTEIN

Overview

Diamond-Blackfan anemia (DBA) is a rare congenital disease linked to, mutations in the ribosomal protein genes rps19, rps24 and rps17. It, belongs to the emerging class of ribosomal disorders. To understand the, impact of DBA mutations on RPS19 function, we have solved the crystal, structure of RPS19 from Pyrococcus abyssi. The protein forms a five, alpha-helix bundle organized around a central amphipathic alpha-helix, which corresponds to the DBA mutation hot spot. From the structure, we, classify DBA mutations relative to their respective impact on protein, folding (class I) or on surface properties (class II). Class II mutations, cluster into two conserved basic patches. In vivo analysis in yeast, demonstrates an essential role for class II residues in the incorporation, into pre-40S ... [(full description)]

About this Structure

2V7F is a [Single protein] structure of sequence from [Pyrococcus abyssi] with CL as [ligand]. Full crystallographic information is available from [OCA].

Reference

Molecular basis of Diamond Blackfan anemia: structure and function analysis of RPS19., Gregory LA, Aguissa-Toure AH, Pinaud N, Legrand P, Gleizes PE, Fribourg S, Nucleic Acids Res. 2007 Aug 28;. PMID:17726054

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