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2pfi

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Revision as of 15:22, 17 June 2021

Crystal structure of the cytoplasmic domain of the human chloride channel ClC-Ka

Structural highlights

2pfi is a 2 chain structure with sequence from Human. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Ligands:	,
Gene:	CLCNKA (HUMAN)
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

[CLCKA_HUMAN] Defects in CLCNKA are a cause of Bartter syndrome type 4B (BS4B) [MIM:613090]. A digenic, recessive disorder characterized by impaired salt reabsorption in the thick ascending loop of Henle with pronounced salt wasting, hypokalemic metabolic alkalosis, and varying degrees of hypercalciuria. Bartter syndrome type 4B is associated with sensorineural deafness.^[1] ^[2]

Function

[CLCKA_HUMAN] Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. May be important in urinary concentrating mechanisms.

Evolutionary Conservation

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

Publication Abstract from PubMed

The cytoplasmic domains of ClC chloride channels and transporters are ubiquitously found in eukaryotic family members and have been suggested to be involved in the regulation of ion transport. All cytoplasmic ClC domains share a conserved scaffold that contains a pair of CBS motifs. Here we describe the structure of the cytoplasmic component of the human chloride channel ClC-Ka at 1.6 A resolution. The structure reveals a dimeric organization of the domain that is unusual for CBS motif containing proteins. Using a biochemical approach combining mutagenesis, crosslinking, and analytical ultracentrifugation, we demonstrate that the interaction interface is preserved in solution and that the distantly related channel ClC-0 likely exhibits a similar structural organization. Our results reveal a conserved interaction interface that relates the cytoplasmic domains of ClC proteins and establish a structural relationship that is likely general for this important family of transport proteins.

The structure of the cytoplasmic domain of the chloride channel ClC-Ka reveals a conserved interaction interface.,Markovic S, Dutzler R Structure. 2007 Jun;15(6):715-25. PMID:17562318^[3]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

References

↑ Nozu K, Inagaki T, Fu XJ, Nozu Y, Kaito H, Kanda K, Sekine T, Igarashi T, Nakanishi K, Yoshikawa N, Iijima K, Matsuo M. Molecular analysis of digenic inheritance in Bartter syndrome with sensorineural deafness. J Med Genet. 2008 Mar;45(3):182-6. doi: 10.1136/jmg.2007.052944. PMID:18310267 doi:10.1136/jmg.2007.052944
↑ Schlingmann KP, Konrad M, Jeck N, Waldegger P, Reinalter SC, Holder M, Seyberth HW, Waldegger S. Salt wasting and deafness resulting from mutations in two chloride channels. N Engl J Med. 2004 Mar 25;350(13):1314-9. PMID:15044642 doi:10.1056/NEJMoa032843
↑ Markovic S, Dutzler R. The structure of the cytoplasmic domain of the chloride channel ClC-Ka reveals a conserved interaction interface. Structure. 2007 Jun;15(6):715-25. PMID:17562318 doi:http://dx.doi.org/10.1016/j.str.2007.04.013

1 Structural highlights
2 Disease
3 Function
4 Evolutionary Conservation
5 Publication Abstract from PubMed
6 See Also
7 References

Proteopedia Page Contributors and Editors (what is this?)

OCA

Retrieved from "http://52.214.119.220/wiki/index.php/2pfi"

Categories: Human | Large Structures | Dutzler, R | Markovic, S | Transport protein

@@ Line 1: / Line 1: @@
 ==Crystal structure of the cytoplasmic domain of the human chloride channel ClC-Ka==
-<StructureSection load='2pfi' size='340' side='right' caption='[[2pfi]], [[Resolution|resolution]] 1.60&Aring;' scene=''>
+<StructureSection load='2pfi' size='340' side='right'caption='[[2pfi]], [[Resolution|resolution]] 1.60&Aring;' scene=''>
 == Structural highlights ==
-<table><tr><td colspan='2'>[[2pfi]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2PFI OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2PFI FirstGlance]. <br>
+<table><tr><td colspan='2'>[[2pfi]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2PFI OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2PFI FirstGlance]. <br>
-</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=IOD:IODIDE+ION'>IOD</scene></td></tr>
+</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=IOD:IODIDE+ION'>IOD</scene></td></tr>
-<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">CLCNKA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
+<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">CLCNKA ([https://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
-<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2pfi FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2pfi OCA], [http://pdbe.org/2pfi PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2pfi RCSB], [http://www.ebi.ac.uk/pdbsum/2pfi PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2pfi ProSAT]</span></td></tr>
+<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2pfi FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2pfi OCA], [https://pdbe.org/2pfi PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2pfi RCSB], [https://www.ebi.ac.uk/pdbsum/2pfi PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2pfi ProSAT]</span></td></tr>
 </table>
 == Disease ==
-[[http://www.uniprot.org/uniprot/CLCKA_HUMAN CLCKA_HUMAN]] Defects in CLCNKA are a cause of Bartter syndrome type 4B (BS4B) [MIM:[http://omim.org/entry/613090 613090]]. A digenic, recessive disorder characterized by impaired salt reabsorption in the thick ascending loop of Henle with pronounced salt wasting, hypokalemic metabolic alkalosis, and varying degrees of hypercalciuria. Bartter syndrome type 4B is associated with sensorineural deafness.<ref>PMID:18310267</ref> <ref>PMID:15044642</ref>
+[[https://www.uniprot.org/uniprot/CLCKA_HUMAN CLCKA_HUMAN]] Defects in CLCNKA are a cause of Bartter syndrome type 4B (BS4B) [MIM:[https://omim.org/entry/613090 613090]]. A digenic, recessive disorder characterized by impaired salt reabsorption in the thick ascending loop of Henle with pronounced salt wasting, hypokalemic metabolic alkalosis, and varying degrees of hypercalciuria. Bartter syndrome type 4B is associated with sensorineural deafness.<ref>PMID:18310267</ref> <ref>PMID:15044642</ref>
 == Function ==
-[[http://www.uniprot.org/uniprot/CLCKA_HUMAN CLCKA_HUMAN]] Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. May be important in urinary concentrating mechanisms.
+[[https://www.uniprot.org/uniprot/CLCKA_HUMAN CLCKA_HUMAN]] Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. May be important in urinary concentrating mechanisms.
 == Evolutionary Conservation ==
 [[Image:Consurf_key_small.gif|200px|right]]
@@ Line 33: / Line 33: @@
 ==See Also==
-*[[Ion channels|Ion channels]]
+*[[Ion channels 3D structures|Ion channels 3D structures]]
 == References ==
 <references/>
@@ Line 39: / Line 39: @@
 </StructureSection>
 [[Category: Human]]
+[[Category: Large Structures]]
 [[Category: Dutzler, R]]
 [[Category: Markovic, S]]
 [[Category: Transport protein]]

2pfi

From Proteopedia

Revision as of 15:22, 17 June 2021

Crystal structure of the cytoplasmic domain of the human chloride channel ClC-Ka

Structural highlights

Disease

Function

Evolutionary Conservation

Publication Abstract from PubMed

See Also

References

Contents

Proteopedia Page Contributors and Editors (what is this?)

Views

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