2wp3 - Revision history

OCA at 08:03, 23 October 2024

2024-10-23T08:03:12Z

← Older revision		Revision as of 08:03, 23 October 2024
Line 17:		Line 17:
	<jmolCheckbox>		<jmolCheckbox>
	<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/wp/2wp3_consurf.spt"</scriptWhenChecked>		<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/wp/2wp3_consurf.spt"</scriptWhenChecked>
	<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/~~initialview01~~.spt</scriptWhenUnchecked>		<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview03.spt</scriptWhenUnchecked>
	<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/main_output.php?pdb_ID=2wp3 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=2wp3 ConSurf].
	<div style="clear:both"></div>		<div style="clear:both"></div>
			<div style="background-color:#fffaf0;">
			== Publication Abstract from PubMed ==
			In the sarcomeric M-band, the giant ruler proteins titin and obscurin, its small homologue obscurin-like-1 (obsl1), and the myosin cross-linking protein myomesin form a ternary complex that is crucial for the function of the M-band as a mechanical link. Mutations in the last titin immunoglobulin (Ig) domain M10, which interacts with the N-terminal Ig-domains of obscurin and obsl1, lead to hereditary muscle diseases. The M10 domain is unusual not only in that it is a frequent target of disease-linked mutations, but also in that it is the only currently known muscle Ig-domain that interacts with two ligands-obscurin and obsl1-in different sarcomeric subregions. Using x-ray crystallography, we show the structural basis for titin M10 interaction with obsl1 in a novel antiparallel Ig-Ig architecture and unravel the molecular basis of titin-M10 linked myopathies. The severity of these pathologies correlates with the disruption of the titin-obsl1/obscurin complex. Conserved signature residues at the interface account for differences in affinity that direct the cellular sorting in cardiomyocytes. By engineering the interface signature residues of obsl1 to obscurin, and vice versa, their affinity for titin can be modulated similar to the native proteins. In single-molecule force-spectroscopy experiments, both complexes yield at forces of around 30 pN, much lower than those observed for the mechanically stable Z-disk complex of titin and telethonin, suggesting why even moderate weakening of the obsl1/obscurin-titin links has severe consequences for normal muscle functions.

			Structural insight into M-band assembly and mechanics from the titin-obscurin-like-1 complex.,Pernigo S, Fukuzawa A, Bertz M, Holt M, Rief M, Steiner RA, Gautel M Proc Natl Acad Sci U S A. 2010 Feb 1. PMID:20133654<ref>PMID:20133654</ref>

			From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
			</div>
			<div class="pdbe-citations 2wp3" style="background-color:#fffaf0;"></div>

	==See Also==		==See Also==

OCA at 07:10, 1 May 2024

2024-05-01T07:10:28Z

← Older revision		Revision as of 07:10, 1 May 2024
Line 3:		Line 3:
	<StructureSection load='2wp3' size='340' side='right'caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>		<StructureSection load='2wp3' size='340' side='right'caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[2wp3]] 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=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>		<table><tr><td colspan='2'>[[2wp3]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>
	</td></tr><tr id='~~ligand~~'><td class="sblockLbl"><b>[[~~Ligand~~\|~~Ligands~~:]]</b></td><td class="sblockDat" id="~~ligandDat~~">~~<scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>~~, ~~<scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>~~</td></tr>		</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models\|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution\|Resolution]] 1.48Å</td></tr>
	<tr id='~~related~~'><td class="sblockLbl"><b>[[~~Related_structure~~\|~~Related~~:]]</b></td><td class="sblockDat"><~~div style~~='~~overflow~~: ~~auto; max-height: 3em;~~'>[[1tnn\|1tnn]], [[1ncu\|1ncu]], [[2j8o\|2j8o]], [[1bpv\|1bpv]], [[2cpc\|2cpc]], [[2j8h\|2j8h]], [[1nct\|1nct]], [[2wwk\|2wwk]], [[2bk8\|2bk8]], [[1tiu\|1tiu]], [[2wwm\|2wwm]], [[2f8v\|2f8v]], [[1tit\|1tit]], [[2a38\|2a38]], [[1tnm\|1tnm]], [[1waa\|1waa]], [[1g1c\|1g1c]], [[1tki\|1tki]], [[1ya5\|1ya5]]</~~div~~><~~/td></tr>~~		<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
	~~<tr id~~='~~activity'><td class~~=~~"sblockLbl"><b>Activity~~:~~</b></td><td class="sblockDat"><span class='plainlinks~~'>[https://en.wikipedia.org/wiki/Non-specific_serine/threonine_protein_kinase Non-specific serine/threonine protein kinase], with EC number [https://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.1 2.7.11.1] </~~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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [https://pdbe.org/2wp3 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [https://www.ebi.ac.uk/pdbsum/2wp3 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2wp3 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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [https://pdbe.org/2wp3 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [https://www.ebi.ac.uk/pdbsum/2wp3 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2wp3 ProSAT]</span></td></tr>
	</table>		</table>
			== Disease ==
			[https://www.uniprot.org/uniprot/OBSL1_HUMAN OBSL1_HUMAN] Defects in OBSL1 are the cause of 3M syndrome type 2 (3M2) [MIM:[https://omim.org/entry/612921 612921]. An autosomal recessive disorder characterized by severe pre- and postnatal growth retardation, facial dysmorphism, large head circumference, and normal intelligence and endocrine function. Skeletal changes include long slender tubular bones and tall vertebral bodies.<ref>PMID:19481195</ref>
			== Function ==
			[https://www.uniprot.org/uniprot/OBSL1_HUMAN OBSL1_HUMAN]
	== Evolutionary Conservation ==		== Evolutionary Conservation ==
	[[Image:Consurf_key_small.gif\|200px\|right]]		[[Image:Consurf_key_small.gif\|200px\|right]]
Line 19:		Line 22:
	</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=2wp3 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=2wp3 ConSurf].
	<div style="clear:both"></div>		<div style="clear:both"></div>
	~~<div style="background-color:#fffaf0;">~~
	~~== Publication Abstract from PubMed ==~~
	In the sarcomeric M-band, the giant ruler proteins titin and obscurin, its small homologue obscurin-like-1 (obsl1), and the myosin cross-linking protein myomesin form a ternary complex that is crucial for the function of the M-band as a mechanical link. Mutations in the last titin immunoglobulin (Ig) domain M10, which interacts with the N-terminal Ig-domains of obscurin and obsl1, lead to hereditary muscle diseases. The M10 domain is unusual not only in that it is a frequent target of disease-linked mutations, but also in that it is the only currently known muscle Ig-domain that interacts with two ligands-obscurin and obsl1-in different sarcomeric subregions. Using x-ray crystallography, we show the structural basis for titin M10 interaction with obsl1 in a novel antiparallel Ig-Ig architecture and unravel the molecular basis of titin-M10 linked myopathies. The severity of these pathologies correlates with the disruption of the titin-obsl1/obscurin complex. Conserved signature residues at the interface account for differences in affinity that direct the cellular sorting in cardiomyocytes. By engineering the interface signature residues of obsl1 to obscurin, and vice versa, their affinity for titin can be modulated similar to the native proteins. In single-molecule force-spectroscopy experiments, both complexes yield at forces of around 30 pN, much lower than those observed for the mechanically stable Z-disk complex of titin and telethonin, suggesting why even moderate weakening of the obsl1/obscurin-titin links has severe consequences for normal muscle functions.

	Structural insight into M-band assembly and mechanics from the titin-obscurin-like-1 complex.,Pernigo S, Fukuzawa A, Bertz M, Holt M, Rief M, Steiner RA, Gautel M Proc Natl Acad Sci U S A. 2010 Feb 1. PMID:20133654<ref>PMID:20133654</ref>

	~~From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>~~
	~~</div>~~
	~~<div class="pdbe-citations 2wp3" style="background-color:#fffaf0;"></div>~~

	==See Also==		==See Also==
	*[[Obscurin\|Obscurin]]		*[[Obscurin\|Obscurin]]
	*[[Titin\|Titin]]		*[[Titin 3D structures\|Titin 3D structures]]
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
	[[Category: ~~Human~~]]		[[Category: Homo sapiens]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
	~~[[Category: Non-specific serine/threonine protein kinase]]~~		[[Category: Fuzukawa A]]
	[[Category: Fuzukawa, A]]		[[Category: Gautel M]]
	[[Category: Gautel, M]]		[[Category: Pernigo S]]
	[[Category: Pernigo, S]]		[[Category: Steiner RA]]
	[[Category: Steiner~~, R A]]~~
	~~[[Category: Atp-binding]]~~
	~~[[Category: Calmodulin-binding]]~~
	~~[[Category: Cardiomyopathy]]~~
	~~[[Category: Immunoglobulin domain]]~~
	~~[[Category: Kelch repeat]]~~
	~~[[Category: Limb-girdle muscular dystrophy]]~~
	~~[[Category: Sarcomere]]~~
	~~[[Category: Serine/threonine-protein kinase]]~~
	~~[[Category: Transferase-structural protein complex~~]]

OCA at 11:12, 6 April 2022

2022-04-06T11:12:44Z

← Older revision		Revision as of 11:12, 6 April 2022
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	<StructureSection load='2wp3' size='340' side='right'caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>		<StructureSection load='2wp3' size='340' side='right'caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[2wp3]] 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=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://proteopedia.org/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>		<table><tr><td colspan='2'>[[2wp3]] 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=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>		</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[1tnn\|1tnn]], [[1ncu\|1ncu]], [[2j8o\|2j8o]], [[1bpv\|1bpv]], [[2cpc\|2cpc]], [[2j8h\|2j8h]], [[1nct\|1nct]], [[2wwk\|2wwk]], [[2bk8\|2bk8]], [[1tiu\|1tiu]], [[2wwm\|2wwm]], [[2f8v\|2f8v]], [[1tit\|1tit]], [[2a38\|2a38]], [[1tnm\|1tnm]], [[1waa\|1waa]], [[1g1c\|1g1c]], [[1tki\|1tki]], [[1ya5\|1ya5]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat"><div style='overflow: auto; max-height: 3em;'>[[1tnn\|1tnn]], [[1ncu\|1ncu]], [[2j8o\|2j8o]], [[1bpv\|1bpv]], [[2cpc\|2cpc]], [[2j8h\|2j8h]], [[1nct\|1nct]], [[2wwk\|2wwk]], [[2bk8\|2bk8]], [[1tiu\|1tiu]], [[2wwm\|2wwm]], [[2f8v\|2f8v]], [[1tit\|1tit]], [[2a38\|2a38]], [[1tnm\|1tnm]], [[1waa\|1waa]], [[1g1c\|1g1c]], [[1tki\|1tki]], [[1ya5\|1ya5]]</div></td></tr>
	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[~~http~~://en.wikipedia.org/wiki/Non-specific_serine/threonine_protein_kinase Non-specific serine/threonine protein kinase], with EC number [~~http~~://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.1 2.7.11.1] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[https://en.wikipedia.org/wiki/Non-specific_serine/threonine_protein_kinase Non-specific serine/threonine protein kinase], with EC number [https://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.1 2.7.11.1] </span></td></tr>
	<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[~~http~~://proteopedia.org/fgij/fg.htm?mol=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [~~http~~://pdbe.org/2wp3 PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/2wp3 PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=2wp3 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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [https://pdbe.org/2wp3 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [https://www.ebi.ac.uk/pdbsum/2wp3 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2wp3 ProSAT]</span></td></tr>
	</table>		</table>
	== Evolutionary Conservation ==		== Evolutionary Conservation ==

OCA at 09:00, 1 July 2020

2020-07-01T09:00:52Z

← Older revision		Revision as of 09:00, 1 July 2020
Line 1:		Line 1:

	==Crystal structure of the Titin M10-Obscurin like 1 Ig complex==		==Crystal structure of the Titin M10-Obscurin like 1 Ig complex==
	<StructureSection load='2wp3' size='340' side='right' caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>		<StructureSection load='2wp3' size='340' side='right'caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[2wp3]] 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=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [http://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>		<table><tr><td colspan='2'>[[2wp3]] 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=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>		</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[1tnn\|1tnn]], [[1ncu\|1ncu]], [[2j8o\|2j8o]], [[1bpv\|1bpv]], [[2cpc\|2cpc]], [[2j8h\|2j8h]], [[1nct\|1nct]], [[2wwk\|2wwk]], [[2bk8\|2bk8]], [[1tiu\|1tiu]], [[2wwm\|2wwm]], [[2f8v\|2f8v]], [[1tit\|1tit]], [[2a38\|2a38]], [[1tnm\|1tnm]], [[1waa\|1waa]], [[1g1c\|1g1c]], [[1tki\|1tki]], [[1ya5\|1ya5]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[1tnn\|1tnn]], [[1ncu\|1ncu]], [[2j8o\|2j8o]], [[1bpv\|1bpv]], [[2cpc\|2cpc]], [[2j8h\|2j8h]], [[1nct\|1nct]], [[2wwk\|2wwk]], [[2bk8\|2bk8]], [[1tiu\|1tiu]], [[2wwm\|2wwm]], [[2f8v\|2f8v]], [[1tit\|1tit]], [[2a38\|2a38]], [[1tnm\|1tnm]], [[1waa\|1waa]], [[1g1c\|1g1c]], [[1tki\|1tki]], [[1ya5\|1ya5]]</td></tr>
	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Non-specific_serine/threonine_protein_kinase Non-specific serine/threonine protein kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.1 2.7.11.1] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Non-specific_serine/threonine_protein_kinase Non-specific serine/threonine protein kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.1 2.7.11.1] </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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [http://pdbe.org/2wp3 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [http://www.ebi.ac.uk/pdbsum/2wp3 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2wp3 ProSAT]</span></td></tr>		<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/fgij/fg.htm?mol=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [http://pdbe.org/2wp3 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [http://www.ebi.ac.uk/pdbsum/2wp3 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2wp3 ProSAT]</span></td></tr>
	</table>		</table>
	== Evolutionary Conservation ==		== Evolutionary Conservation ==
Line 37:		Line 37:
	</StructureSection>		</StructureSection>
	[[Category: Human]]		[[Category: Human]]
			[[Category: Large Structures]]
	[[Category: Non-specific serine/threonine protein kinase]]		[[Category: Non-specific serine/threonine protein kinase]]
	[[Category: Fuzukawa, A]]		[[Category: Fuzukawa, A]]

OCA at 09:15, 26 September 2018

2018-09-26T09:15:07Z

← Older revision		Revision as of 09:15, 26 September 2018
Line 1:		Line 1:

	==~~CRYSTAL STRUCTURE OF THE TITIN~~ M10-~~OBSCURIN LIKE~~ 1 ~~IG COMPLEX~~==		==Crystal structure of the Titin M10-Obscurin like 1 Ig complex==
	<StructureSection load='2wp3' size='340' side='right' caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>		<StructureSection load='2wp3' size='340' side='right' caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
Line 9:		Line 9:
	<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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [http://pdbe.org/2wp3 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [http://www.ebi.ac.uk/pdbsum/2wp3 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2wp3 ProSAT]</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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [http://pdbe.org/2wp3 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [http://www.ebi.ac.uk/pdbsum/2wp3 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2wp3 ProSAT]</span></td></tr>
	</table>		</table>
	~~== Disease ==~~
	[[http://www.uniprot.org/uniprot/OBSL1_HUMAN OBSL1_HUMAN]] Defects in OBSL1 are the cause of 3M syndrome type 2 (3M2) [MIM:[http://omim.org/entry/612921 612921]]. An autosomal recessive disorder characterized by severe pre- and postnatal growth retardation, facial dysmorphism, large head circumference, and normal intelligence and endocrine function. Skeletal changes include long slender tubular bones and tall vertebral bodies.<ref>PMID:19481195</ref>
	== Evolutionary Conservation ==		== Evolutionary Conservation ==
	[[Image:Consurf_key_small.gif\|200px\|right]]		[[Image:Consurf_key_small.gif\|200px\|right]]
	Check<jmol>		Check<jmol>
	<jmolCheckbox>		<jmolCheckbox>
	<scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/wp/2wp3_consurf.spt"</scriptWhenChecked>		<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/wp/2wp3_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 11:02, 5 August 2016

2016-08-05T11:02:24Z

← Older revision		Revision as of 11:02, 5 August 2016
Line 1:		Line 1:

	==CRYSTAL STRUCTURE OF THE TITIN M10-OBSCURIN LIKE 1 IG COMPLEX==		==CRYSTAL STRUCTURE OF THE TITIN M10-OBSCURIN LIKE 1 IG COMPLEX==
	<StructureSection load='2wp3' size='340' side='right' caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>		<StructureSection load='2wp3' size='340' side='right' caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[2wp3]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/~~Homo_sapiens Homo sapiens~~]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>		<table><tr><td colspan='2'>[[2wp3]] 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=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>		</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[1tnn\|1tnn]], [[1ncu\|1ncu]], [[2j8o\|2j8o]], [[1bpv\|1bpv]], [[2cpc\|2cpc]], [[2j8h\|2j8h]], [[1nct\|1nct]], [[2wwk\|2wwk]], [[2bk8\|2bk8]], [[1tiu\|1tiu]], [[2wwm\|2wwm]], [[2f8v\|2f8v]], [[1tit\|1tit]], [[2a38\|2a38]], [[1tnm\|1tnm]], [[1waa\|1waa]], [[1g1c\|1g1c]], [[1tki\|1tki]], [[1ya5\|1ya5]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[1tnn\|1tnn]], [[1ncu\|1ncu]], [[2j8o\|2j8o]], [[1bpv\|1bpv]], [[2cpc\|2cpc]], [[2j8h\|2j8h]], [[1nct\|1nct]], [[2wwk\|2wwk]], [[2bk8\|2bk8]], [[1tiu\|1tiu]], [[2wwm\|2wwm]], [[2f8v\|2f8v]], [[1tit\|1tit]], [[2a38\|2a38]], [[1tnm\|1tnm]], [[1waa\|1waa]], [[1g1c\|1g1c]], [[1tki\|1tki]], [[1ya5\|1ya5]]</td></tr>
	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Non-specific_serine/threonine_protein_kinase Non-specific serine/threonine protein kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.1 2.7.11.1] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Non-specific_serine/threonine_protein_kinase Non-specific serine/threonine protein kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.1 2.7.11.1] </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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [http://www.ebi.ac.uk/pdbsum/2wp3 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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [http://pdbe.org/2wp3 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [http://www.ebi.ac.uk/pdbsum/2wp3 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2wp3 ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 18:		Line 19:
	<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=2wp3 ConSurf].
	<div style="clear:both"></div>		<div style="clear:both"></div>
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
Line 28:		Line 29:
	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 2wp3" style="background-color:#fffaf0;"></div>

	==See Also==		==See Also==
Line 36:		Line 38:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
	[[Category: ~~Homo sapiens~~]]		[[Category: Human]]
	[[Category: Non-specific serine/threonine protein kinase]]		[[Category: Non-specific serine/threonine protein kinase]]
	[[Category: Fuzukawa, A]]		[[Category: Fuzukawa, A]]

OCA at 18:15, 25 December 2014

2014-12-25T18:15:47Z

← Older revision		Revision as of 18:15, 25 December 2014
Line 3:		Line 3:
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[2wp3]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>		<table><tr><td colspan='2'>[[2wp3]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>
	</td></tr><tr><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene><br>		</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
	<tr><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[1tnn\|1tnn]], [[1ncu\|1ncu]], [[2j8o\|2j8o]], [[1bpv\|1bpv]], [[2cpc\|2cpc]], [[2j8h\|2j8h]], [[1nct\|1nct]], [[2wwk\|2wwk]], [[2bk8\|2bk8]], [[1tiu\|1tiu]], [[2wwm\|2wwm]], [[2f8v\|2f8v]], [[1tit\|1tit]], [[2a38\|2a38]], [[1tnm\|1tnm]], [[1waa\|1waa]], [[1g1c\|1g1c]], [[1tki\|1tki]], [[1ya5\|1ya5]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[1tnn\|1tnn]], [[1ncu\|1ncu]], [[2j8o\|2j8o]], [[1bpv\|1bpv]], [[2cpc\|2cpc]], [[2j8h\|2j8h]], [[1nct\|1nct]], [[2wwk\|2wwk]], [[2bk8\|2bk8]], [[1tiu\|1tiu]], [[2wwm\|2wwm]], [[2f8v\|2f8v]], [[1tit\|1tit]], [[2a38\|2a38]], [[1tnm\|1tnm]], [[1waa\|1waa]], [[1g1c\|1g1c]], [[1tki\|1tki]], [[1ya5\|1ya5]]</td></tr>
	<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Non-specific_serine/threonine_protein_kinase Non-specific serine/threonine protein kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.1 2.7.11.1] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Non-specific_serine/threonine_protein_kinase Non-specific serine/threonine protein kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.1 2.7.11.1] </span></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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [http://www.ebi.ac.uk/pdbsum/2wp3 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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [http://www.ebi.ac.uk/pdbsum/2wp3 PDBsum]</span></td></tr>
	<table>		</table>
	== Disease ==		== Disease ==
	[[http://www.uniprot.org/uniprot/OBSL1_HUMAN OBSL1_HUMAN]] Defects in OBSL1 are the cause of 3M syndrome type 2 (3M2) [MIM:[http://omim.org/entry/612921 612921]]. An autosomal recessive disorder characterized by severe pre- and postnatal growth retardation, facial dysmorphism, large head circumference, and normal intelligence and endocrine function. Skeletal changes include long slender tubular bones and tall vertebral bodies.<ref>PMID:19481195</ref>		[[http://www.uniprot.org/uniprot/OBSL1_HUMAN OBSL1_HUMAN]] Defects in OBSL1 are the cause of 3M syndrome type 2 (3M2) [MIM:[http://omim.org/entry/612921 612921]]. An autosomal recessive disorder characterized by severe pre- and postnatal growth retardation, facial dysmorphism, large head circumference, and normal intelligence and endocrine function. Skeletal changes include long slender tubular bones and tall vertebral bodies.<ref>PMID:19481195</ref>
	~~== Function ==~~

	== Evolutionary Conservation ==		== Evolutionary Conservation ==
	[[Image:Consurf_key_small.gif\|200px\|right]]		[[Image:Consurf_key_small.gif\|200px\|right]]
Line 40:		Line 38:
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Non-specific serine/threonine protein kinase]]		[[Category: Non-specific serine/threonine protein kinase]]
	[[Category: Fuzukawa, A.]]		[[Category: Fuzukawa, A]]
	[[Category: Gautel, M.]]		[[Category: Gautel, M]]
	[[Category: Pernigo, S.]]		[[Category: Pernigo, S]]
	[[Category: Steiner, R A.]]		[[Category: Steiner, R A]]
	[[Category: Atp-binding]]		[[Category: Atp-binding]]
	[[Category: Calmodulin-binding]]		[[Category: Calmodulin-binding]]

OCA at 01:25, 1 October 2014

2014-10-01T01:25:41Z

← Older revision		Revision as of 01:25, 1 October 2014
Line 1:		Line 1:
	~~{{STRUCTURE_2wp3\| PDB=2wp3 \| SCENE= }}~~		==CRYSTAL STRUCTURE OF THE TITIN M10-OBSCURIN LIKE 1 IG COMPLEX==
	===CRYSTAL STRUCTURE OF THE TITIN M10-OBSCURIN LIKE 1 IG COMPLEX===		<StructureSection load='2wp3' size='340' side='right' caption='[[2wp3]], [[Resolution\|resolution]] 1.48Å' scene=''>
	~~{{ABSTRACT_PUBMED_20133654}}~~		== Structural highlights ==
			<table><tr><td colspan='2'>[[2wp3]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2WP3 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2WP3 FirstGlance]. <br>
			</td></tr><tr><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene><br>
			<tr><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[1tnn\|1tnn]], [[1ncu\|1ncu]], [[2j8o\|2j8o]], [[1bpv\|1bpv]], [[2cpc\|2cpc]], [[2j8h\|2j8h]], [[1nct\|1nct]], [[2wwk\|2wwk]], [[2bk8\|2bk8]], [[1tiu\|1tiu]], [[2wwm\|2wwm]], [[2f8v\|2f8v]], [[1tit\|1tit]], [[2a38\|2a38]], [[1tnm\|1tnm]], [[1waa\|1waa]], [[1g1c\|1g1c]], [[1tki\|1tki]], [[1ya5\|1ya5]]</td></tr>
			<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Non-specific_serine/threonine_protein_kinase Non-specific serine/threonine protein kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.11.1 2.7.11.1] </span></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=2wp3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2wp3 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2wp3 RCSB], [http://www.ebi.ac.uk/pdbsum/2wp3 PDBsum]</span></td></tr>
			<table>
			== Disease ==
			[[http://www.uniprot.org/uniprot/OBSL1_HUMAN OBSL1_HUMAN]] Defects in OBSL1 are the cause of 3M syndrome type 2 (3M2) [MIM:[http://omim.org/entry/612921 612921]]. An autosomal recessive disorder characterized by severe pre- and postnatal growth retardation, facial dysmorphism, large head circumference, and normal intelligence and endocrine function. Skeletal changes include long slender tubular bones and tall vertebral bodies.<ref>PMID:19481195</ref>
			== Function ==

	==~~Disease~~==		== Evolutionary Conservation ==
	[[http://~~www~~.~~uniprot~~.~~org~~/~~uniprot/OBSL1_HUMAN OBSL1_HUMAN~~]] ~~Defects in OBSL1 are~~ the ~~cause of 3M syndrome type 2 (3M2) [MIM:~~[http://~~omim~~.~~org~~/~~entry~~/~~612921 612921]~~]. ~~An autosomal recessive disorder characterized by severe pre~~- and ~~postnatal growth retardation~~, ~~facial dysmorphism~~, ~~large head circumference~~, and ~~normal intelligence~~ and ~~endocrine function~~. ~~Skeletal changes include long slender tubular bones~~ and ~~tall vertebral bodies~~.~~<ref>PMID:19481195<~~/~~ref>~~		[[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/wp/2wp3_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 ==
			In the sarcomeric M-band, the giant ruler proteins titin and obscurin, its small homologue obscurin-like-1 (obsl1), and the myosin cross-linking protein myomesin form a ternary complex that is crucial for the function of the M-band as a mechanical link. Mutations in the last titin immunoglobulin (Ig) domain M10, which interacts with the N-terminal Ig-domains of obscurin and obsl1, lead to hereditary muscle diseases. The M10 domain is unusual not only in that it is a frequent target of disease-linked mutations, but also in that it is the only currently known muscle Ig-domain that interacts with two ligands-obscurin and obsl1-in different sarcomeric subregions. Using x-ray crystallography, we show the structural basis for titin M10 interaction with obsl1 in a novel antiparallel Ig-Ig architecture and unravel the molecular basis of titin-M10 linked myopathies. The severity of these pathologies correlates with the disruption of the titin-obsl1/obscurin complex. Conserved signature residues at the interface account for differences in affinity that direct the cellular sorting in cardiomyocytes. By engineering the interface signature residues of obsl1 to obscurin, and vice versa, their affinity for titin can be modulated similar to the native proteins. In single-molecule force-spectroscopy experiments, both complexes yield at forces of around 30 pN, much lower than those observed for the mechanically stable Z-disk complex of titin and telethonin, suggesting why even moderate weakening of the obsl1/obscurin-titin links has severe consequences for normal muscle functions.

	~~==About this Structure==~~		Structural insight into M-band assembly and mechanics from the titin-obscurin-like-1 complex.,Pernigo S, Fukuzawa A, Bertz M, Holt M, Rief M, Steiner RA, Gautel M Proc Natl Acad Sci U S A. 2010 Feb 1. PMID:20133654<ref>PMID:20133654</ref>
	~~[[2wp3]] is a 2 chain structure with sequence~~ from ~~[http://en~~.~~wikipedia~~.~~org/wiki/Homo_sapiens Homo sapiens]~~. ~~Full crystallographic information is available from [http~~://~~oca~~.~~weizmann~~.ac.il/~~oca-bin/ocashort?id=2WP3 OCA].~~
			From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
			</div>

	==See Also==		==See Also==
	*[[Obscurin\|Obscurin]]		*[[Obscurin\|Obscurin]]
	*[[Titin\|Titin]]		*[[Titin\|Titin]]
			== References ==
	==~~Reference~~==		<references/>
	~~<ref group="xtra">PMID:020133654</ref>~~<references ~~group="xtra"~~/><~~references~~/>		__TOC__
			</StructureSection>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Non-specific serine/threonine protein kinase]]		[[Category: Non-specific serine/threonine protein kinase]]

OCA at 20:52, 24 March 2013

2013-03-24T20:52:59Z

← Older revision		Revision as of 20:52, 24 March 2013
Line 1:		Line 1:
	~~[[Image:2wp3.png\|left\|200px]]~~

	{{STRUCTURE_2wp3\| PDB=2wp3 \| SCENE= }}		{{STRUCTURE_2wp3\| PDB=2wp3 \| SCENE= }}

	===CRYSTAL STRUCTURE OF THE TITIN M10-OBSCURIN LIKE 1 IG COMPLEX===		===CRYSTAL STRUCTURE OF THE TITIN M10-OBSCURIN LIKE 1 IG COMPLEX===
			{{ABSTRACT_PUBMED_20133654}}

	~~{{ABSTRACT_PUBMED_20133654}}~~		==Disease==
			[[http://www.uniprot.org/uniprot/OBSL1_HUMAN OBSL1_HUMAN]] Defects in OBSL1 are the cause of 3M syndrome type 2 (3M2) [MIM:[http://omim.org/entry/612921 612921]]. An autosomal recessive disorder characterized by severe pre- and postnatal growth retardation, facial dysmorphism, large head circumference, and normal intelligence and endocrine function. Skeletal changes include long slender tubular bones and tall vertebral bodies.<ref>PMID:19481195</ref>

	==About this Structure==		==About this Structure==
Line 15:		Line 14:

	==Reference==		==Reference==
	<ref group="xtra">PMID:020133654</ref><references group="xtra"/>		<ref group="xtra">PMID:020133654</ref><references group="xtra"/><references/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Non-specific serine/threonine protein kinase]]		[[Category: Non-specific serine/threonine protein kinase]]

OCA at 19:52, 20 October 2012

2012-10-20T19:52:10Z

← Older revision		Revision as of 19:52, 20 October 2012
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	==About this Structure==		==About this Structure==
	[[2wp3]] is a 2 chain structure ~~of [[Obscurin]] and [[Titin]]~~ with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2WP3 OCA].		[[2wp3]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2WP3 OCA].

	==See Also==		==See Also==