2bx6
From Proteopedia
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| - | [[Image:2bx6.gif|left|200px]]<br /> | + | [[Image:2bx6.gif|left|200px]]<br /><applet load="2bx6" size="450" color="white" frame="true" align="right" spinBox="true" |
| - | <applet load="2bx6" size="450" color="white" frame="true" align="right" spinBox="true" | + | |
caption="2bx6, resolution 2.1Å" /> | caption="2bx6, resolution 2.1Å" /> | ||
'''CRYSTAL STRUCTURE OF THE HUMAN RETINITIS PIGMENTOSA PROTEIN 2 (RP2)'''<br /> | '''CRYSTAL STRUCTURE OF THE HUMAN RETINITIS PIGMENTOSA PROTEIN 2 (RP2)'''<br /> | ||
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==About this Structure== | ==About this Structure== | ||
| - | 2BX6 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with SO4 as [http://en.wikipedia.org/wiki/ligand ligand]. | + | 2BX6 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with SO4 as [http://en.wikipedia.org/wiki/ligand ligand]. Known structural/functional Site: <scene name='pdbsite=AC1:So4 Binding Site For Chain A'>AC1</scene>. Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=2BX6 OCA]. |
==Reference== | ==Reference== | ||
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[[Category: vision]] | [[Category: vision]] | ||
| - | ''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on | + | ''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Tue Dec 18 19:01:34 2007'' |
Revision as of 16:51, 18 December 2007
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CRYSTAL STRUCTURE OF THE HUMAN RETINITIS PIGMENTOSA PROTEIN 2 (RP2)
Contents |
Overview
The crystal structure of human retinitis pigmentosa 2 protein (RP2) was, solved to 2.1 angstroms resolution. It consists of an N-terminal beta, helix and a C-terminal ferredoxin-like alpha/beta domain. RP2 is, functionally and structurally related to the tubulin-specific chaperone, cofactor C. Seven of nine known RP2 missense mutations identified in, patients are located in the beta helix domain, and most of them cluster to, the hydrophobic core and are likely to destabilize the protein. Two, residues, Glu138 and the catalytically important Arg118, are, solvent-exposed and form a salt bridge, indicating that Glu138 might be, critical for positioning Arg118 for catalysis. RP2 is a specific effector, protein of Arl3. The N-terminal 34 residues and beta helix domain of RP2, are required for this interaction. The abilitities of RP2 to bind Arl3 and, cause retinitis pigmentosa seem to be correlated, since both the R118H and, E138G mutants show a drastically reduced affinity to Arl3.
Disease
Known diseases associated with this structure: Retinitis pigmentosa-2 OMIM:[312600], Xp11.3 deletion syndrome OMIM:[312600]
About this Structure
2BX6 is a Single protein structure of sequence from Homo sapiens with SO4 as ligand. Known structural/functional Site: . Full crystallographic information is available from OCA.
Reference
Crystal structure of the human retinitis pigmentosa 2 protein and its interaction with Arl3., Kuhnel K, Veltel S, Schlichting I, Wittinghofer A, Structure. 2006 Feb;14(2):367-78. PMID:16472755
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