2per

Crystal Structure of Human Chloride Intracellular Channel protein 2Crystal Structure of Human Chloride Intracellular Channel protein 2

Structural highlights

2per is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:	X-ray diffraction, Resolution 2Å
Ligands:
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

CLIC2_HUMAN Note=Defects in CLIC2 are a cause of a mental retardation with cardiopathy and seizures. Cardiac features include atrial fibrillation, cardiomegaly, and congestive heart failure.

Function

CLIC2_HUMAN Can insert into membranes and form chloride ion channels. Channel activity depends on the pH. Membrane insertion seems to be redox-regulated and may occur only under oxydizing conditions. Modulates the activity of RYR2 and inhibits calcium influx.^[1] ^[2] ^[3]

Evolutionary Conservation

Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf.

References

↑ Board PG, Coggan M, Watson S, Gage PW, Dulhunty AF. CLIC-2 modulates cardiac ryanodine receptor Ca2+ release channels. Int J Biochem Cell Biol. 2004 Aug;36(8):1599-612. PMID:15147738 doi:10.1016/j.biocel.2004.01.026
↑ Dulhunty AF, Pouliquin P, Coggan M, Gage PW, Board PG. A recently identified member of the glutathione transferase structural family modifies cardiac RyR2 substate activity, coupled gating and activation by Ca2+ and ATP. Biochem J. 2005 Aug 15;390(Pt 1):333-43. PMID:15916532 doi:BJ20042113
↑ Cromer BA, Gorman MA, Hansen G, Adams JJ, Coggan M, Littler DR, Brown LJ, Mazzanti M, Breit SN, Curmi PM, Dulhunty AF, Board PG, Parker MW. Structure of the Janus protein human CLIC2. J Mol Biol. 2007 Nov 30;374(3):719-31. Epub 2007 Sep 20. PMID:17945253 doi:http://dx.doi.org/10.1016/j.jmb.2007.09.041

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OCA

[1] Board PG, Coggan M, Watson S, Gage PW, Dulhunty AF. CLIC-2 modulates cardiac ryanodine receptor Ca2+ release channels. Int J Biochem Cell Biol. 2004 Aug;36(8):1599-612. PMID:15147738 doi:10.1016/j.biocel.2004.01.026

[2] Dulhunty AF, Pouliquin P, Coggan M, Gage PW, Board PG. A recently identified member of the glutathione transferase structural family modifies cardiac RyR2 substate activity, coupled gating and activation by Ca2+ and ATP. Biochem J. 2005 Aug 15;390(Pt 1):333-43. PMID:15916532 doi:BJ20042113

[3] Cromer BA, Gorman MA, Hansen G, Adams JJ, Coggan M, Littler DR, Brown LJ, Mazzanti M, Breit SN, Curmi PM, Dulhunty AF, Board PG, Parker MW. Structure of the Janus protein human CLIC2. J Mol Biol. 2007 Nov 30;374(3):719-31. Epub 2007 Sep 20. PMID:17945253 doi:http://dx.doi.org/10.1016/j.jmb.2007.09.041

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