2v7f: Difference between revisions

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[[Image:2v7f.jpg|left|200px]]
{{Seed}}
[[Image:2v7f.png|left|200px]]


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{{STRUCTURE_2v7f|  PDB=2v7f  |  SCENE=  }}  
{{STRUCTURE_2v7f|  PDB=2v7f  |  SCENE=  }}  


'''STRUCTURE OF P. ABYSSI RPS19 PROTEIN'''
===STRUCTURE OF P. ABYSSI RPS19 PROTEIN===




==Overview==
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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 ribosomal particles. This data indicate that missense mutations in DBA primarily affect the capacity of the protein to be incorporated into pre-ribosomes, thus blocking maturation of the pre-40S particles.
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{{ABSTRACT_PUBMED_17726054}}


==About this Structure==
==About this Structure==
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[[Category: Diamond blackfan anemia small ribosomal subunit]]
[[Category: Diamond blackfan anemia small ribosomal subunit]]
[[Category: Ribosomal protein]]
[[Category: Ribosomal protein]]
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun May  4 18:19:21 2008''
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jul 28 23:19:38 2008''

Revision as of 20:19, 28 July 2008

File:2v7f.png

Template:STRUCTURE 2v7f

STRUCTURE OF P. ABYSSI RPS19 PROTEIN

Template:ABSTRACT PUBMED 17726054

About this Structure

2V7F is a Single protein structure of sequence from Pyrococcus abyssi. 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;35(17):5913-21. Epub 2007 Aug 28. PMID:17726054

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