2hrf - Revision history

OCA at 19:02, 29 May 2024

OCA — Wed, 29 May 2024 19:02:57 GMT

←Older revision		Revision as of 19:02, 29 May 2024
Line 1:		Line 1:

	==Solution Structure of Cu(I) P174L HSco1==		==Solution Structure of Cu(I) P174L HSco1==
-	<StructureSection load='2hrf' size='340' side='right'caption='[[2hrf~~]], [[NMR_Ensembles_of_Models \| 1 NMR models~~]]' scene=''>	+	<StructureSection load='2hrf' size='340' side='right'caption='[[2hrf]]' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[2hrf]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/~~Human Human~~]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2HRF OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2HRF FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[2hrf]] 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=2HRF OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2HRF FirstGlance]. <br>
-	</td></tr><tr id='~~ligand~~'><td class="sblockLbl"><b>[[~~Ligand~~\|~~Ligands~~:]]</b></td><td class="sblockDat" id="~~ligandDat~~">~~<scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene>~~</td></tr>	+	</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models\|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
-	<tr id='~~related~~'><td class="sblockLbl"><b>[[~~Related_structure~~\|~~Related~~:]]</b></td><td class="sblockDat"~~><div style~~=~~'overflow: auto; max-height: 3em;'~~>~~[[2gqk\|2gqk]], [[2gql\|2gql]], [[2gqm\|2gqm]], [[2gt6\|2gt6]], [[2gt5\|2gt5]], [[2gvp\|2gvp]], [[2hrn\|2hrn]]~~<~~/div></td></tr>~~	+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene></td></tr>
-	~~<tr id~~='~~gene'><td class~~=~~"sblockLbl"><b>[[Gene\|Gene~~:~~]]</b~~></td>~~<td class="sblockDat">SCO1, SCOD1 ([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'>[https://proteopedia.org/fgij/fg.htm?mol=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [https://pdbe.org/2hrf PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [https://www.ebi.ac.uk/pdbsum/2hrf PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2hrf 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=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [https://pdbe.org/2hrf PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [https://www.ebi.ac.uk/pdbsum/2hrf PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2hrf ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
-	[[https://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:[https://omim.org/entry/220110 220110]]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.<ref>PMID:17189203</ref> <ref>PMID:11013136</ref>	+	[https://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN] Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:[https://omim.org/entry/220110 220110]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.<ref>PMID:17189203</ref> <ref>PMID:11013136</ref>
	== Function ==		== Function ==
-	[[https://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX.<ref>PMID:17189203</ref> <ref>PMID:15659396</ref> <ref>PMID:16735468</ref>	+	[https://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN] Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX.<ref>PMID:17189203</ref> <ref>PMID:15659396</ref> <ref>PMID:16735468</ref>
	== Evolutionary Conservation ==		== Evolutionary Conservation ==
	[[Image:Consurf_key_small.gif\|200px\|right]]		[[Image:Consurf_key_small.gif\|200px\|right]]
Line 36:		Line 35:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Human~~]]	+	[[Category: Homo sapiens]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	[[Category: Banci, L]]	+	[[Category: Banci L]]
-	[[Category: Bertini, I]]	+	[[Category: Bertini I]]
-	[[Category: Ciofi-Baffoni, S]]	+	[[Category: Ciofi-Baffoni S]]
-	[[Category: Leontari, I]]	+	[[Category: Leontari I]]
-	[[Category: Martinelli, M]]	+	[[Category: Martinelli M]]
-	[[Category: Palumaa, P]]	+	[[Category: Palumaa P]]
-	~~[[Category: SPINE, Structural Proteomics in Europe~~]]	+	[[Category: Sillard R]]
-	[[Category: Sillard, R]]	+	[[Category: Wang S]]
-	[[Category: Wang, S]]	+
-	~~[[Category: Chaperone]]~~	+
-	~~[[Category: Disease mutation]]~~	+
-	~~[[Category: Metal transport]]~~	+
-	~~[[Category: Metal-binding]]~~	+
-	~~[[Category: Mitochondrion]]~~	+
-	~~[[Category: Spine]]~~	+
-	~~[[Category: Structural genomic]]~~	+
-	~~[[Category: Structural proteomics in europe~~]]	+

OCA at 07:20, 24 March 2021

OCA — Wed, 24 Mar 2021 07:20:33 GMT

←Older revision		Revision as of 07:20, 24 March 2021
Line 1:		Line 1:

	==Solution Structure of Cu(I) P174L HSco1==		==Solution Structure of Cu(I) P174L HSco1==
-	<StructureSection load='2hrf' size='340' side='right' caption='[[2hrf]], [[NMR_Ensembles_of_Models \| 1 NMR models]]' scene=''>	+	<StructureSection load='2hrf' size='340' side='right'caption='[[2hrf]], [[NMR_Ensembles_of_Models \| 1 NMR models]]' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[2hrf]] 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=2HRF OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=2HRF FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[2hrf]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2HRF OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2HRF FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene></td></tr>	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene></td></tr>
-	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2gqk\|2gqk]], [[2gql\|2gql]], [[2gqm\|2gqm]], [[2gt6\|2gt6]], [[2gt5\|2gt5]], [[2gvp\|2gvp]], [[2hrn\|2hrn]]</td></tr>	+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat"><div style='overflow: auto; max-height: 3em;'>[[2gqk\|2gqk]], [[2gql\|2gql]], [[2gqm\|2gqm]], [[2gt6\|2gt6]], [[2gt5\|2gt5]], [[2gvp\|2gvp]], [[2hrn\|2hrn]]</div></td></tr>
-	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">SCO1, SCOD1 ([~~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">SCO1, SCOD1 ([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=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [~~http~~://pdbe.org/2hrf PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/2hrf PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=2hrf 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=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [https://pdbe.org/2hrf PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [https://www.ebi.ac.uk/pdbsum/2hrf PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2hrf ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
-	[[~~http~~://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:[~~http~~://omim.org/entry/220110 220110]]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.<ref>PMID:17189203</ref> <ref>PMID:11013136</ref>	+	[[https://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:[https://omim.org/entry/220110 220110]]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.<ref>PMID:17189203</ref> <ref>PMID:11013136</ref>
	== Function ==		== Function ==
-	[[~~http~~://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX.<ref>PMID:17189203</ref> <ref>PMID:15659396</ref> <ref>PMID:16735468</ref>	+	[[https://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX.<ref>PMID:17189203</ref> <ref>PMID:15659396</ref> <ref>PMID:16735468</ref>
	== Evolutionary Conservation ==		== Evolutionary Conservation ==
	[[Image:Consurf_key_small.gif\|200px\|right]]		[[Image:Consurf_key_small.gif\|200px\|right]]
Line 37:		Line 37:
	</StructureSection>		</StructureSection>
	[[Category: Human]]		[[Category: Human]]
		+	[[Category: Large Structures]]
	[[Category: Banci, L]]		[[Category: Banci, L]]
	[[Category: Bertini, I]]		[[Category: Bertini, I]]

OCA at 06:36, 27 June 2018

OCA — Wed, 27 Jun 2018 06:36:52 GMT

←Older revision		Revision as of 06:36, 27 June 2018
Line 1:		Line 1:
		+
	==Solution Structure of Cu(I) P174L HSco1==		==Solution Structure of Cu(I) P174L HSco1==
	<StructureSection load='2hrf' size='340' side='right' caption='[[2hrf]], [[NMR_Ensembles_of_Models \| 1 NMR models]]' scene=''>		<StructureSection load='2hrf' size='340' side='right' caption='[[2hrf]], [[NMR_Ensembles_of_Models \| 1 NMR models]]' scene=''>
Line 6:		Line 7:
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2gqk\|2gqk]], [[2gql\|2gql]], [[2gqm\|2gqm]], [[2gt6\|2gt6]], [[2gt5\|2gt5]], [[2gvp\|2gvp]], [[2hrn\|2hrn]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2gqk\|2gqk]], [[2gql\|2gql]], [[2gqm\|2gqm]], [[2gt6\|2gt6]], [[2gt5\|2gt5]], [[2gvp\|2gvp]], [[2hrn\|2hrn]]</td></tr>
	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">SCO1, SCOD1 ([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">SCO1, SCOD1 ([http://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=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [http://pdbe.org/2hrf PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [http://www.ebi.ac.uk/pdbsum/2hrf PDBsum]</span></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=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [http://pdbe.org/2hrf PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [http://www.ebi.ac.uk/pdbsum/2hrf PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2hrf ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 16:		Line 17:
	Check<jmol>		Check<jmol>
	<jmolCheckbox>		<jmolCheckbox>
-	<scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/hr/2hrf_consurf.spt"</scriptWhenChecked>	+	<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/hr/2hrf_consurf.spt"</scriptWhenChecked>
	<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>		<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
	<text>to colour the structure by Evolutionary Conservation</text>		<text>to colour the structure by Evolutionary Conservation</text>

OCA at 00:01, 10 February 2016

OCA — Wed, 10 Feb 2016 00:01:45 GMT

←Older revision		Revision as of 00:01, 10 February 2016
Line 20:		Line 20:
	<text>to colour the structure by Evolutionary Conservation</text>		<text>to colour the structure by Evolutionary Conservation</text>
	</jmolCheckbox>		</jmolCheckbox>
-	</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary\|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/~~chain_selection~~.php?pdb_ID=~~2ata~~ ConSurf].	+	</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary\|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=2hrf ConSurf].
	<div style="clear:both"></div>		<div style="clear:both"></div>
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">

OCA at 03:21, 11 September 2015

OCA — Fri, 11 Sep 2015 03:21:51 GMT

←Older revision		Revision as of 03:21, 11 September 2015
Line 2:		Line 2:
	<StructureSection load='2hrf' size='340' side='right' caption='[[2hrf]], [[NMR_Ensembles_of_Models \| 1 NMR models]]' scene=''>		<StructureSection load='2hrf' size='340' side='right' caption='[[2hrf]], [[NMR_Ensembles_of_Models \| 1 NMR models]]' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[2hrf]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/~~Homo_sapiens Homo sapiens~~]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2HRF OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2HRF FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[2hrf]] 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=2HRF OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2HRF FirstGlance]. <br>
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene></td></tr>		</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2gqk\|2gqk]], [[2gql\|2gql]], [[2gqm\|2gqm]], [[2gt6\|2gt6]], [[2gt5\|2gt5]], [[2gvp\|2gvp]], [[2hrn\|2hrn]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2gqk\|2gqk]], [[2gql\|2gql]], [[2gqm\|2gqm]], [[2gt6\|2gt6]], [[2gt5\|2gt5]], [[2gvp\|2gvp]], [[2hrn\|2hrn]]</td></tr>
-	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">SCO1, SCOD1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 ~~Homo sapiens~~])</td></tr>	+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">SCO1, SCOD1 ([http://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=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [http://www.ebi.ac.uk/pdbsum/2hrf PDBsum]</span></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=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [http://pdbe.org/2hrf PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [http://www.ebi.ac.uk/pdbsum/2hrf PDBsum]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 30:		Line 30:
	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>		From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
	</div>		</div>
		+	<div class="pdbe-citations 2hrf" style="background-color:#fffaf0;"></div>
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Homo sapiens~~]]	+	[[Category: Human]]
	[[Category: Banci, L]]		[[Category: Banci, L]]
	[[Category: Bertini, I]]		[[Category: Bertini, I]]

OCA at 08:24, 16 January 2015

OCA — Fri, 16 Jan 2015 08:24:51 GMT

←Older revision		Revision as of 08:24, 16 January 2015
Line 3:		Line 3:
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[2hrf]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2HRF OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2HRF FirstGlance]. <br>		<table><tr><td colspan='2'>[[2hrf]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2HRF OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2HRF FirstGlance]. <br>
-	</td></tr><tr><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene><br>	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene></td></tr>
-	<tr><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2gqk\|2gqk]], [[2gql\|2gql]], [[2gqm\|2gqm]], [[2gt6\|2gt6]], [[2gt5\|2gt5]], [[2gvp\|2gvp]], [[2hrn\|2hrn]]</td></tr>	+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2gqk\|2gqk]], [[2gql\|2gql]], [[2gqm\|2gqm]], [[2gt6\|2gt6]], [[2gt5\|2gt5]], [[2gvp\|2gvp]], [[2hrn\|2hrn]]</td></tr>
-	<tr><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">SCO1, SCOD1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>	+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">SCO1, SCOD1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
-	<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [http://www.ebi.ac.uk/pdbsum/2hrf PDBsum]</span></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=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [http://www.ebi.ac.uk/pdbsum/2hrf PDBsum]</span></td></tr>
-	<table>	+	</table>
	== Disease ==		== Disease ==
	[[http://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:[http://omim.org/entry/220110 220110]]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.<ref>PMID:17189203</ref> <ref>PMID:11013136</ref>		[[http://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:[http://omim.org/entry/220110 220110]]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.<ref>PMID:17189203</ref> <ref>PMID:11013136</ref>
Line 35:		Line 35:
	</StructureSection>		</StructureSection>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
-	[[Category: Banci, L.]]	+	[[Category: Banci, L]]
-	[[Category: Bertini, I.]]	+	[[Category: Bertini, I]]
-	[[Category: Ciofi-Baffoni, S.]]	+	[[Category: Ciofi-Baffoni, S]]
-	[[Category: Leontari, I.]]	+	[[Category: Leontari, I]]
-	[[Category: Martinelli, M.]]	+	[[Category: Martinelli, M]]
-	[[Category: Palumaa, P.]]	+	[[Category: Palumaa, P]]
-	[[Category: SPINE, Structural Proteomics in Europe.]]	+	[[Category: SPINE, Structural Proteomics in Europe]]
-	[[Category: Sillard, R.]]	+	[[Category: Sillard, R]]
-	[[Category: Wang, S.]]	+	[[Category: Wang, S]]
	[[Category: Chaperone]]		[[Category: Chaperone]]
	[[Category: Disease mutation]]		[[Category: Disease mutation]]

OCA at 09:35, 30 September 2014

OCA — Tue, 30 Sep 2014 09:35:41 GMT

←Older revision		Revision as of 09:35, 30 September 2014
Line 1:		Line 1:
-	~~{{STRUCTURE_2hrf~~\| ~~PDB~~=2hrf \| ~~SCENE~~= }}	+	==Solution Structure of Cu(I) P174L HSco1==
-	===~~Solution Structure~~ of Cu(I) P174L HSco1~~===~~	+	<StructureSection load='2hrf' size='340' side='right' caption='[[2hrf]], [[NMR_Ensembles_of_Models \| 1 NMR models]]' scene=''>
-	~~{{ABSTRACT_PUBMED_17182746}}~~	+	== Structural highlights ==
		+	<table><tr><td colspan='2'>[[2hrf]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2HRF OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2HRF FirstGlance]. <br>
		+	</td></tr><tr><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene><br>
		+	<tr><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2gqk\|2gqk]], [[2gql\|2gql]], [[2gqm\|2gqm]], [[2gt6\|2gt6]], [[2gt5\|2gt5]], [[2gvp\|2gvp]], [[2hrn\|2hrn]]</td></tr>
		+	<tr><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">SCO1, SCOD1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
		+	<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2hrf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hrf OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2hrf RCSB], [http://www.ebi.ac.uk/pdbsum/2hrf PDBsum]</span></td></tr>
		+	<table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:[http://omim.org/entry/220110 220110]]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.<ref>PMID:17189203</ref> <ref>PMID:11013136</ref>
		+	== Function ==
		+	[[http://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX.<ref>PMID:17189203</ref> <ref>PMID:15659396</ref> <ref>PMID:16735468</ref>
		+	== Evolutionary Conservation ==
		+	[[Image:Consurf_key_small.gif\|200px\|right]]
		+	Check<jmol>
		+	<jmolCheckbox>
		+	<scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/hr/2hrf_consurf.spt"</scriptWhenChecked>
		+	<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
		+	<text>to colour the structure by Evolutionary Conservation</text>
		+	</jmolCheckbox>
		+	</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary\|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/chain_selection.php?pdb_ID=2ata ConSurf].
		+	<div style="clear:both"></div>
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	The pathogenic mutant (P174L) of human Sco1 produces respiratory chain deficiency associated with cytochrome c oxidase (CcO) assembly defects. The solution structure of the mutant in its Cu(I) form shows that Leu-174 prevents the formation of a well packed hydrophobic region around the metal-binding site and causes a reduction of the affinity of copper(I) for the protein. K(D) values for Cu(I)WT-HSco1 and Cu(I)P174L-HSco1 are approximately 10(-17) and approximately 10(-13), respectively. The reduction potentials of the two apo proteins are similar, but slower reduction/oxidation rates are found for the mutant with respect to the WT. The mitochondrial metallochaperone in the partially oxidized Cu(1)(I)Cox17(2S-S) form, at variance with the fully reduced Cu(4)(I)Cox17, interacts transiently with both WT-HSco1 and the mutant, forming the Cox17/Cu(I)/HSco1 complex, but copper is efficiently transferred only in the case of WT protein. Cu(1)(I)Cox17(2S-S) indeed has an affinity for copper(I) (K(D) approximately 10(-15)) higher than that of the P174L-HSco1 mutant but lower than that of WT-HSco1. We propose that HSco1 mutation, altering the structure around the metal-binding site, affects both copper(I) binding and redox properties of the protein, thus impairing the efficiency of copper transfer to CcO. The pathogenic mutation therefore could (i) lessen the Sco1 affinity for copper(I) and hence copper supply for CcO or (ii) decrease the efficiency of reduction of CcO thiols involved in copper binding, or both effects could be produced by the mutation.

-	~~==Disease==~~	+	Human Sco1 functional studies and pathological implications of the P174L mutant.,Banci L, Bertini I, Ciofi-Baffoni S, Leontari I, Martinelli M, Palumaa P, Sillard R, Wang S Proc Natl Acad Sci U S A. 2007 Jan 2;104(1):15-20. Epub 2006 Dec 20. PMID:17182746<ref>PMID:17182746</ref>
-	[[http://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:[http://omim.org/entry/220110 220110]]; also known as cytochrome c oxidase deficiency. A disorder of the ~~mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs~~. ~~Features include hypertrophic cardiomyopathy~~, ~~hepatomegaly and liver dysfunction~~, ~~hypotonia~~, ~~muscle weakness~~, ~~excercise intolerance~~, ~~developmental delay~~, ~~delayed motor development and mental retardation.~~ A ~~subset of patients manifest Leigh syndrome~~.~~<ref>~~PMID:~~17189203</ref>~~<ref>PMID:~~11013136~~</ref>	+

-	~~==Function==~~	+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
-	~~[[http:~~/~~/www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Thought to play~~ a ~~role in cellular copper homeostasis, mitochondrial redox signaling or insertion~~ of ~~copper into~~ the ~~active site~~ of ~~COX~~.<~~ref~~>~~PMID:17189203~~</~~ref~~>~~<ref>PMID:15659396</ref><ref>PMID:16735468</ref>~~	+	</div>
-		+	== References ==
-	==~~About this Structure==~~	+	<references/>
-	[[2hrf]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2HRF OCA].	+	__TOC__
-		+	</StructureSection>
-	~~==Reference~~==	+
-	~~<ref group="xtra">PMID:017182746</ref>~~<references ~~group="xtra"~~/><~~references~~/>	+
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Banci, L.]]		[[Category: Banci, L.]]

OCA at 21:04, 24 March 2013

OCA — Sun, 24 Mar 2013 21:04:44 GMT

←Older revision		Revision as of 21:04, 24 March 2013
Line 1:		Line 1:
-	[[Image:2hrf.png\|left\|200px]]
-
	{{STRUCTURE_2hrf\| PDB=2hrf \| SCENE= }}		{{STRUCTURE_2hrf\| PDB=2hrf \| SCENE= }}
-
	===Solution Structure of Cu(I) P174L HSco1===		===Solution Structure of Cu(I) P174L HSco1===
		+	{{ABSTRACT_PUBMED_17182746}}

-	~~{{ABSTRACT_PUBMED_17182746}}~~	+	==Disease==
		+	[[http://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:[http://omim.org/entry/220110 220110]]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.<ref>PMID:17189203</ref><ref>PMID:11013136</ref>
		+
		+	==Function==
		+	[[http://www.uniprot.org/uniprot/SCO1_HUMAN SCO1_HUMAN]] Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX.<ref>PMID:17189203</ref><ref>PMID:15659396</ref><ref>PMID:16735468</ref>

	==About this Structure==		==About this Structure==
Line 11:		Line 13:

	==Reference==		==Reference==
-	<ref group="xtra">PMID:017182746</ref><references group="xtra"/>	+	<ref group="xtra">PMID:017182746</ref><references group="xtra"/><references/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Banci, L.]]		[[Category: Banci, L.]]

OCA: Protected "2hrf" [edit=sysop:move=sysop]

OCA — Mon, 07 Jan 2013 14:30:04 GMT

Protected "2hrf" [edit=sysop:move=sysop]

←Older revision

Revision as of 14:30, 7 January 2013

OCA at 14:30, 7 January 2013

OCA — Mon, 07 Jan 2013 14:30:02 GMT

←Older revision		Revision as of 14:30, 7 January 2013
Line 1:		Line 1:
-	{{Seed}}
	[[Image:2hrf.png\|left\|200px]]		[[Image:2hrf.png\|left\|200px]]

-	<!--
-	The line below this paragraph, containing "STRUCTURE_2hrf", creates the "Structure Box" on the page.
-	You may change the PDB parameter (which sets the PDB file loaded into the applet)
-	or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
-	or leave the SCENE parameter empty for the default display.
-	-->
	{{STRUCTURE_2hrf\| PDB=2hrf \| SCENE= }}		{{STRUCTURE_2hrf\| PDB=2hrf \| SCENE= }}

	===Solution Structure of Cu(I) P174L HSco1===		===Solution Structure of Cu(I) P174L HSco1===

-
-	<!--
-	The line below this paragraph, {{ABSTRACT_PUBMED_17182746}}, adds the Publication Abstract to the page
-	(as it appears on PubMed at http://www.pubmed.gov), where 17182746 is the PubMed ID number.
-	-->
	{{ABSTRACT_PUBMED_17182746}}		{{ABSTRACT_PUBMED_17182746}}

	==About this Structure==		==About this Structure==
-	~~2HRF~~ is a 1 chain structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2HRF OCA].	+	[[2hrf]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2HRF OCA].

	==Reference==		==Reference==
-	<ref group="xtra">PMID:~~17182746~~</ref><references group="xtra"/>	+	<ref group="xtra">PMID:017182746</ref><references group="xtra"/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Banci, L.]]		[[Category: Banci, L.]]
Line 34:		Line 22:
	[[Category: Sillard, R.]]		[[Category: Sillard, R.]]
	[[Category: Wang, S.]]		[[Category: Wang, S.]]
-	[[Category: 3d-structure]]
	[[Category: Chaperone]]		[[Category: Chaperone]]
	[[Category: Disease mutation]]		[[Category: Disease mutation]]
		+	[[Category: Metal transport]]
	[[Category: Metal-binding]]		[[Category: Metal-binding]]
	[[Category: Mitochondrion]]		[[Category: Mitochondrion]]
Line 42:		Line 30:
	[[Category: Structural genomic]]		[[Category: Structural genomic]]
	[[Category: Structural proteomics in europe]]		[[Category: Structural proteomics in europe]]
-
-	''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Feb 17 13:47:54 2009''