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| | ==Solution structure of CHCHD7== | | ==Solution structure of CHCHD7== |
| - | <StructureSection load='2lqt' size='340' side='right' caption='[[2lqt]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''> | + | <StructureSection load='2lqt' size='340' side='right'caption='[[2lqt]]' scene=''> |
| | == Structural highlights == | | == Structural highlights == |
| - | <table><tr><td colspan='2'>[[2lqt]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2LQT OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2LQT FirstGlance]. <br> | + | <table><tr><td colspan='2'>[[2lqt]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2LQT OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2LQT FirstGlance]. <br> |
| - | </td></tr><tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2lql|2lql]]</td></tr> | + | </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=2lqt FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2lqt OCA], [https://pdbe.org/2lqt PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2lqt RCSB], [https://www.ebi.ac.uk/pdbsum/2lqt PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2lqt ProSAT]</span></td></tr> |
| - | <tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">CHCHD7 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
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| - | <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=2lqt FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2lqt OCA], [http://pdbe.org/2lqt PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2lqt RCSB], [http://www.ebi.ac.uk/pdbsum/2lqt PDBsum]</span></td></tr> | + | |
| | </table> | | </table> |
| | == Disease == | | == Disease == |
| - | [[http://www.uniprot.org/uniprot/CHCH7_HUMAN CHCH7_HUMAN]] A chromosomal aberration involving CHCHD7 is found in salivary gland pleiomorphic adenomas, the most common benign epithelial tumors of the salivary gland. Translocation t(6;8)(p21.3-22;q13) with PLAG1. | + | [https://www.uniprot.org/uniprot/CHCH7_HUMAN CHCH7_HUMAN] A chromosomal aberration involving CHCHD7 is found in salivary gland pleiomorphic adenomas, the most common benign epithelial tumors of the salivary gland. Translocation t(6;8)(p21.3-22;q13) with PLAG1. |
| | + | == Function == |
| | + | [https://www.uniprot.org/uniprot/CHCH7_HUMAN CHCH7_HUMAN] |
| | <div style="background-color:#fffaf0;"> | | <div style="background-color:#fffaf0;"> |
| | == Publication Abstract from PubMed == | | == Publication Abstract from PubMed == |
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| | __TOC__ | | __TOC__ |
| | </StructureSection> | | </StructureSection> |
| - | [[Category: Human]] | + | [[Category: Homo sapiens]] |
| - | [[Category: Banci, L]] | + | [[Category: Large Structures]] |
| - | [[Category: Bertini, I]] | + | [[Category: Banci L]] |
| - | [[Category: Ciofi-Baffoni, S]] | + | [[Category: Bertini I]] |
| - | [[Category: Winkelmann, J]] | + | [[Category: Ciofi-Baffoni S]] |
| - | [[Category: Alpha-hairpin]]
| + | [[Category: Winkelmann J]] |
| - | [[Category: Chch domain]]
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| - | [[Category: Mia40-dependent disulfide relay system]]
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| - | [[Category: Mitochondrial import]]
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| - | [[Category: Unknown function]]
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| Structural highlights
Disease
CHCH7_HUMAN A chromosomal aberration involving CHCHD7 is found in salivary gland pleiomorphic adenomas, the most common benign epithelial tumors of the salivary gland. Translocation t(6;8)(p21.3-22;q13) with PLAG1.
Function
CHCH7_HUMAN
Publication Abstract from PubMed
Twin CX(9)C proteins constitute a large protein family among all eukaryotes; are putative substrates of the mitochondrial Mia40-dependent import machinery; contain a coiled coil-helix-coiled coil-helix (CHCH) fold stabilized by two disulfide bonds as exemplified by three structures available for this family. However, they considerably differ at the primary sequence level and this prevents an accurate prediction of their structural models. With the aim of expanding structural information on CHCH proteins, here we structurally characterized human CHCHD5 and CHCHD7. While CHCHD5 has two weakly interacting CHCH domains which sample a range of limited conformations as a consequence of hydrophobic interactions, CHCHD7 has a third helix hydrophobically interacting with an extension of helix alpha2, which is part of the CHCH domain. Upon reduction of the disulfide bonds both proteins become unstructured exposing hydrophobic patches, with the result of protein aggregation/precipitation. These results suggest a model where the molecular interactions guiding the protein recognition between Mia40 and the disulfide-reduced CHCHD5 and CHCHD7 substrates occurs in vivo when the latter proteins are partially embedded in the protein import pore of the outer membrane of mitochondria.
Structural characterization of CHCHD5 and CHCHD7: Two atypical human twin CX(9)C proteins.,Banci L, Bertini I, Ciofi-Baffoni S, Jaiswal D, Neri S, Peruzzini R, Winkelmann J J Struct Biol. 2012 Oct;180(1):190-200. doi: 10.1016/j.jsb.2012.07.007. Epub 2012, Jul 25. PMID:22842048[1]
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
References
- ↑ Banci L, Bertini I, Ciofi-Baffoni S, Jaiswal D, Neri S, Peruzzini R, Winkelmann J. Structural characterization of CHCHD5 and CHCHD7: Two atypical human twin CX(9)C proteins. J Struct Biol. 2012 Oct;180(1):190-200. doi: 10.1016/j.jsb.2012.07.007. Epub 2012, Jul 25. PMID:22842048 doi:http://dx.doi.org/10.1016/j.jsb.2012.07.007
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