6nl3: Difference between revisions

Revision as of 15:27, 20 November 2019

Solution structure of human Coa6

Structural highlights

6nl3 is a 1 chain structure. Full experimental information is available from OCA. For a guided tour on the structure components use FirstGlance.
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

[COA6_HUMAN] Fatal infantile cytochrome C oxidase deficiency. The disease is caused by mutations affecting the gene represented in this entry.

Function

[COA6_HUMAN] Involved in the maturation of the mitochondrial respiratory chain complex IV subunit MT-CO2/COX2. Thereby, may regulate early steps of complex IV assembly. Mitochondrial respiratory chain complex IV or cytochrome c oxidase is the component of the respiratory chain that catalyzes the transfer of electrons from intermembrane space cytochrome c to molecular oxygen in the matrix and as a consequence contributes to the proton gradient involved in mitochondrial ATP synthesis. May also be required for efficient formation of respiratory supercomplexes comprised of complexes III and IV.^[1] ^[2] ^[3]

References

↑ Ghosh A, Trivedi PP, Timbalia SA, Griffin AT, Rahn JJ, Chan SS, Gohil VM. Copper supplementation restores cytochrome c oxidase assembly defect in a mitochondrial disease model of COA6 deficiency. Hum Mol Genet. 2014 Jul 1;23(13):3596-606. doi: 10.1093/hmg/ddu069. Epub 2014 Feb, 18. PMID:24549041 doi:https://dx.doi.org/10.1093/hmg/ddu069
↑ Pacheu-Grau D, Bareth B, Dudek J, Juris L, Vogtle FN, Wissel M, Leary SC, Dennerlein S, Rehling P, Deckers M. Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies. Cell Metab. 2015 Jun 2;21(6):823-33. doi: 10.1016/j.cmet.2015.04.012. Epub 2015, May 7. PMID:25959673 doi:https://dx.doi.org/10.1016/j.cmet.2015.04.012
↑ Stroud DA, Maher MJ, Lindau C, Vogtle FN, Frazier AE, Surgenor E, Mountford H, Singh AP, Bonas M, Oeljeklaus S, Warscheid B, Meisinger C, Thorburn DR, Ryan MT. COA6 is a mitochondrial complex IV assembly factor critical for biogenesis of mtDNA-encoded COX2. Hum Mol Genet. 2015 Oct 1;24(19):5404-15. doi: 10.1093/hmg/ddv265. Epub 2015 Jul , 9. PMID:26160915 doi:https://dx.doi.org/10.1093/hmg/ddv265

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OCA

[1] Ghosh A, Trivedi PP, Timbalia SA, Griffin AT, Rahn JJ, Chan SS, Gohil VM. Copper supplementation restores cytochrome c oxidase assembly defect in a mitochondrial disease model of COA6 deficiency. Hum Mol Genet. 2014 Jul 1;23(13):3596-606. doi: 10.1093/hmg/ddu069. Epub 2014 Feb, 18. PMID:24549041 doi:https://dx.doi.org/10.1093/hmg/ddu069

[2] Pacheu-Grau D, Bareth B, Dudek J, Juris L, Vogtle FN, Wissel M, Leary SC, Dennerlein S, Rehling P, Deckers M. Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies. Cell Metab. 2015 Jun 2;21(6):823-33. doi: 10.1016/j.cmet.2015.04.012. Epub 2015, May 7. PMID:25959673 doi:https://dx.doi.org/10.1016/j.cmet.2015.04.012

[3] Stroud DA, Maher MJ, Lindau C, Vogtle FN, Frazier AE, Surgenor E, Mountford H, Singh AP, Bonas M, Oeljeklaus S, Warscheid B, Meisinger C, Thorburn DR, Ryan MT. COA6 is a mitochondrial complex IV assembly factor critical for biogenesis of mtDNA-encoded COX2. Hum Mol Genet. 2015 Oct 1;24(19):5404-15. doi: 10.1093/hmg/ddv265. Epub 2015 Jul , 9. PMID:26160915 doi:https://dx.doi.org/10.1093/hmg/ddv265

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[2]

[3]

@@ Line 1: / Line 1: @@
-'''Unreleased structure'''
-The entry 6nl3 is ON HOLD  until Paper Publication
+==Solution structure of human Coa6==
+<StructureSection load='6nl3' size='340' side='right'caption='[[6nl3]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''>
-Authors:
+== Structural highlights ==
+<table><tr><td colspan='2'>[[6nl3]] is a 1 chain structure. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6NL3 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6NL3 FirstGlance]. <br>
-Description:
+</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=6nl3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6nl3 OCA], [http://pdbe.org/6nl3 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6nl3 RCSB], [http://www.ebi.ac.uk/pdbsum/6nl3 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6nl3 ProSAT]</span></td></tr>
-[[Category: Unreleased Structures]]
+</table>
+== Disease ==
+[[http://www.uniprot.org/uniprot/COA6_HUMAN COA6_HUMAN]] Fatal infantile cytochrome C oxidase deficiency. The disease is caused by mutations affecting the gene represented in this entry.
+== Function ==
+[[http://www.uniprot.org/uniprot/COA6_HUMAN COA6_HUMAN]] Involved in the maturation of the mitochondrial respiratory chain complex IV subunit MT-CO2/COX2. Thereby, may regulate early steps of complex IV assembly. Mitochondrial respiratory chain complex IV or cytochrome c oxidase is the component of the respiratory chain that catalyzes the transfer of electrons from intermembrane space cytochrome c to molecular oxygen in the matrix and as a consequence contributes to the proton gradient involved in mitochondrial ATP synthesis. May also be required for efficient formation of respiratory supercomplexes comprised of complexes III and IV.<ref>PMID:24549041</ref> <ref>PMID:25959673</ref> <ref>PMID:26160915</ref>
+== References ==
+<references/>
+__TOC__
+</StructureSection>
+[[Category: Large Structures]]
+[[Category: Gohil, V]]
+[[Category: Naik, M T]]
+[[Category: Soma, S]]
+[[Category: Mitochondrial protein]]
+[[Category: Oxidoreductase]]

6nl3: Difference between revisions

Revision as of 15:27, 20 November 2019

Solution structure of human Coa6

Structural highlights

Disease

Function

References

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