3pdy - Revision history

OCA at 09:52, 6 September 2023

2023-09-06T09:52:08Z

←Older revision		Revision as of 09:52, 6 September 2023
Line 3:		Line 3:
	<StructureSection load='3pdy' size='340' side='right'caption='[[3pdy]], [[Resolution\|resolution]] 2.22Å' scene=''>		<StructureSection load='3pdy' size='340' side='right'caption='[[3pdy]], [[Resolution\|resolution]] 2.22Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[3pdy]] 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=3PDY OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3PDY FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[3pdy]] 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=3PDY OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3PDY FirstGlance]. <br>
-	</td></tr><tr id='~~ligand~~'><td class="sblockLbl"><b>[[~~Ligand~~\|~~Ligands~~:]]</b></td><td class="sblockDat" id="~~ligandDat~~">~~<scene name='pdbligand=EDO:1~~,2~~-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</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]] 2.2182Å</td></tr>
-	<tr id='~~related~~'><td class="sblockLbl"><b>[[~~Related_structure~~\|~~Related~~:]]</b></td><td class="sblockDat"><~~div style~~='~~overflow~~: ~~auto; max~~-~~height: 3em;~~'>~~[[2odu\|2odu]], [[2odv\|2odv]], [[2iak\|2iak]], [[3pe0\|3pe0]]~~</~~div~~><~~/td></tr>~~	+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</scene></td></tr>
-	~~<tr id~~='~~gene'><td class~~=~~"sblockLbl"><b>[[Gene\|Gene~~:~~]]</b~~></td>~~<td class="sblockDat">PLEC, PLEC1 ([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=3pdy FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3pdy OCA], [https://pdbe.org/3pdy PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3pdy RCSB], [https://www.ebi.ac.uk/pdbsum/3pdy PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3pdy 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=3pdy FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3pdy OCA], [https://pdbe.org/3pdy PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3pdy RCSB], [https://www.ebi.ac.uk/pdbsum/3pdy PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3pdy ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
-	[[https://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Defects in PLEC are the cause of epidermolysis bullosa simplex with pyloric atresia (EBS-PA) [MIM:[https://omim.org/entry/612138 612138]]. EBS-PA is an autosomal recessive genodermatosis characterized by severe skin blistering at birth and congenital pyloric atresia. Death usually occurs in infancy. This disorder is allelic to MD-EBS.<ref>PMID:8698233</ref> <ref>PMID:20665883</ref> <ref>PMID:14675180</ref> Defects in PLEC are the cause of epidermolysis bullosa simplex with muscular dystrophy (MD-EBS) [MIM:[https://omim.org/entry/226670 226670]]. MD-EBS is an autosomal recessive disorder characterized by epidermal blister formation at the level of the hemidesmosome and associated with late-onset muscular dystrophy. Defects in PLEC are the cause of epidermolysis bullosa simplex Ogna type (O-EBS) [MIM:[https://omim.org/entry/131950 131950]]; also called epidermolysis bullosa simplex 1. O-EBS is a form of intraepidermal epidermolysis bullosa characterized by generalized skin bruising, skin fragility with non-scarring blistering and small hemorrhagic blisters on hands. At the ultrastructural level, it is differentiated from classical cases of K-EBS, WC-EBS and DM-EBS, by the occurrence of blisters originating in basal cells above hemidesmosomes, and abnormal hemidesmosome intracellular attachment plates. Defects in PLEC are the cause of limb-girdle muscular dystrophy type 2Q (LGMD2Q) [MIM:[https://omim.org/entry/613723 613723]]. An autosomal recessive degenerative myopathy characterized by early childhood onset of proximal muscle weakness. Note=A 9 bp deletion containing the initiation codon in exon 1f of PLEC have been found in limb-girdle muscular dystrophy patients. The mutation results in deficient expression of isoform 9 and disorganization of the myofibers, without any effect on the skin.<ref>PMID:21109228</ref> <ref>PMID:8698233</ref>	+	[https://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN] Defects in PLEC are the cause of epidermolysis bullosa simplex with pyloric atresia (EBS-PA) [MIM:[https://omim.org/entry/612138 612138]. EBS-PA is an autosomal recessive genodermatosis characterized by severe skin blistering at birth and congenital pyloric atresia. Death usually occurs in infancy. This disorder is allelic to MD-EBS.<ref>PMID:8698233</ref> <ref>PMID:20665883</ref> <ref>PMID:14675180</ref> Defects in PLEC are the cause of epidermolysis bullosa simplex with muscular dystrophy (MD-EBS) [MIM:[https://omim.org/entry/226670 226670]. MD-EBS is an autosomal recessive disorder characterized by epidermal blister formation at the level of the hemidesmosome and associated with late-onset muscular dystrophy. Defects in PLEC are the cause of epidermolysis bullosa simplex Ogna type (O-EBS) [MIM:[https://omim.org/entry/131950 131950]; also called epidermolysis bullosa simplex 1. O-EBS is a form of intraepidermal epidermolysis bullosa characterized by generalized skin bruising, skin fragility with non-scarring blistering and small hemorrhagic blisters on hands. At the ultrastructural level, it is differentiated from classical cases of K-EBS, WC-EBS and DM-EBS, by the occurrence of blisters originating in basal cells above hemidesmosomes, and abnormal hemidesmosome intracellular attachment plates. Defects in PLEC are the cause of limb-girdle muscular dystrophy type 2Q (LGMD2Q) [MIM:[https://omim.org/entry/613723 613723]. An autosomal recessive degenerative myopathy characterized by early childhood onset of proximal muscle weakness. Note=A 9 bp deletion containing the initiation codon in exon 1f of PLEC have been found in limb-girdle muscular dystrophy patients. The mutation results in deficient expression of isoform 9 and disorganization of the myofibers, without any effect on the skin.<ref>PMID:21109228</ref> <ref>PMID:8698233</ref>
	== Function ==		== Function ==
-	[[https://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, but also in the regulation of their dynamics. Structural component of muscle. Isoform 9 plays a major role in the maintenance of myofibers integrity.<ref>PMID:12482924</ref> <ref>PMID:21109228</ref>	+	[https://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN] Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, but also in the regulation of their dynamics. Structural component of muscle. Isoform 9 plays a major role in the maintenance of myofibers integrity.<ref>PMID:12482924</ref> <ref>PMID:21109228</ref>
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
Line 29:		Line 28:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Human~~]]	+	[[Category: Homo sapiens]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	[[Category: Ortega, E]]	+	[[Category: Ortega E]]
-	[[Category: Pereda~~, J M.de]]~~	+	[[Category: De Pereda JM]]
-	~~[[Category: Crosslinking]]~~	+
-	~~[[Category: Cytoskeleton]]~~	+
-	~~[[Category: Intermediate filament]]~~	+
-	~~[[Category: Plakin]]~~	+
-	~~[[Category: Spectrin repeat]]~~	+
-	~~[[Category: Structural protein~~]]	+

OCA at 08:35, 25 May 2022

2022-05-25T08:35:30Z

←Older revision		Revision as of 08:35, 25 May 2022
Line 1:		Line 1:

	==Structure of the third and fourth spectrin repeats of the plakin domain of plectin==		==Structure of the third and fourth spectrin repeats of the plakin domain of plectin==
-	<StructureSection load='3pdy' size='340' side='right' caption='[[3pdy]], [[Resolution\|resolution]] 2.22Å' scene=''>	+	<StructureSection load='3pdy' size='340' side='right'caption='[[3pdy]], [[Resolution\|resolution]] 2.22Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[3pdy]] 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=3PDY OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=3PDY FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[3pdy]] 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=3PDY OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3PDY FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</scene></td></tr>	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</scene></td></tr>
-	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2odu\|2odu]], [[2odv\|2odv]], [[2iak\|2iak]], [[3pe0\|3pe0]]</td></tr>	+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat"><div style='overflow: auto; max-height: 3em;'>[[2odu\|2odu]], [[2odv\|2odv]], [[2iak\|2iak]], [[3pe0\|3pe0]]</div></td></tr>
-	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">PLEC, PLEC1 ([~~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">PLEC, PLEC1 ([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=3pdy FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3pdy OCA], [~~http~~://pdbe.org/3pdy PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=3pdy RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/3pdy PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=3pdy 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=3pdy FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3pdy OCA], [https://pdbe.org/3pdy PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3pdy RCSB], [https://www.ebi.ac.uk/pdbsum/3pdy PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3pdy ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
-	[[~~http~~://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Defects in PLEC are the cause of epidermolysis bullosa simplex with pyloric atresia (EBS-PA) [MIM:[~~http~~://omim.org/entry/612138 612138]]. EBS-PA is an autosomal recessive genodermatosis characterized by severe skin blistering at birth and congenital pyloric atresia. Death usually occurs in infancy. This disorder is allelic to MD-EBS.<ref>PMID:8698233</ref> <ref>PMID:20665883</ref> <ref>PMID:14675180</ref> Defects in PLEC are the cause of epidermolysis bullosa simplex with muscular dystrophy (MD-EBS) [MIM:[~~http~~://omim.org/entry/226670 226670]]. MD-EBS is an autosomal recessive disorder characterized by epidermal blister formation at the level of the hemidesmosome and associated with late-onset muscular dystrophy. Defects in PLEC are the cause of epidermolysis bullosa simplex Ogna type (O-EBS) [MIM:[~~http~~://omim.org/entry/131950 131950]]; also called epidermolysis bullosa simplex 1. O-EBS is a form of intraepidermal epidermolysis bullosa characterized by generalized skin bruising, skin fragility with non-scarring blistering and small hemorrhagic blisters on hands. At the ultrastructural level, it is differentiated from classical cases of K-EBS, WC-EBS and DM-EBS, by the occurrence of blisters originating in basal cells above hemidesmosomes, and abnormal hemidesmosome intracellular attachment plates. Defects in PLEC are the cause of limb-girdle muscular dystrophy type 2Q (LGMD2Q) [MIM:[~~http~~://omim.org/entry/613723 613723]]. An autosomal recessive degenerative myopathy characterized by early childhood onset of proximal muscle weakness. Note=A 9 bp deletion containing the initiation codon in exon 1f of PLEC have been found in limb-girdle muscular dystrophy patients. The mutation results in deficient expression of isoform 9 and disorganization of the myofibers, without any effect on the skin.<ref>PMID:21109228</ref> <ref>PMID:8698233</ref>	+	[[https://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Defects in PLEC are the cause of epidermolysis bullosa simplex with pyloric atresia (EBS-PA) [MIM:[https://omim.org/entry/612138 612138]]. EBS-PA is an autosomal recessive genodermatosis characterized by severe skin blistering at birth and congenital pyloric atresia. Death usually occurs in infancy. This disorder is allelic to MD-EBS.<ref>PMID:8698233</ref> <ref>PMID:20665883</ref> <ref>PMID:14675180</ref> Defects in PLEC are the cause of epidermolysis bullosa simplex with muscular dystrophy (MD-EBS) [MIM:[https://omim.org/entry/226670 226670]]. MD-EBS is an autosomal recessive disorder characterized by epidermal blister formation at the level of the hemidesmosome and associated with late-onset muscular dystrophy. Defects in PLEC are the cause of epidermolysis bullosa simplex Ogna type (O-EBS) [MIM:[https://omim.org/entry/131950 131950]]; also called epidermolysis bullosa simplex 1. O-EBS is a form of intraepidermal epidermolysis bullosa characterized by generalized skin bruising, skin fragility with non-scarring blistering and small hemorrhagic blisters on hands. At the ultrastructural level, it is differentiated from classical cases of K-EBS, WC-EBS and DM-EBS, by the occurrence of blisters originating in basal cells above hemidesmosomes, and abnormal hemidesmosome intracellular attachment plates. Defects in PLEC are the cause of limb-girdle muscular dystrophy type 2Q (LGMD2Q) [MIM:[https://omim.org/entry/613723 613723]]. An autosomal recessive degenerative myopathy characterized by early childhood onset of proximal muscle weakness. Note=A 9 bp deletion containing the initiation codon in exon 1f of PLEC have been found in limb-girdle muscular dystrophy patients. The mutation results in deficient expression of isoform 9 and disorganization of the myofibers, without any effect on the skin.<ref>PMID:21109228</ref> <ref>PMID:8698233</ref>
	== Function ==		== Function ==
-	[[~~http~~://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, but also in the regulation of their dynamics. Structural component of muscle. Isoform 9 plays a major role in the maintenance of myofibers integrity.<ref>PMID:12482924</ref> <ref>PMID:21109228</ref>	+	[[https://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, but also in the regulation of their dynamics. Structural component of muscle. Isoform 9 plays a major role in the maintenance of myofibers integrity.<ref>PMID:12482924</ref> <ref>PMID:21109228</ref>
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
Line 30:		Line 30:
	</StructureSection>		</StructureSection>
	[[Category: Human]]		[[Category: Human]]
		+	[[Category: Large Structures]]
	[[Category: Ortega, E]]		[[Category: Ortega, E]]
	[[Category: Pereda, J M.de]]		[[Category: Pereda, J M.de]]

OCA at 10:59, 5 August 2016

2016-08-05T10:59:36Z

←Older revision		Revision as of 10:59, 5 August 2016
Line 1:		Line 1:
		+
	==Structure of the third and fourth spectrin repeats of the plakin domain of plectin==		==Structure of the third and fourth spectrin repeats of the plakin domain of plectin==
	<StructureSection load='3pdy' size='340' side='right' caption='[[3pdy]], [[Resolution\|resolution]] 2.22Å' scene=''>		<StructureSection load='3pdy' size='340' side='right' caption='[[3pdy]], [[Resolution\|resolution]] 2.22Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[3pdy]] 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=3PDY OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3PDY FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[3pdy]] 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=3PDY OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3PDY FirstGlance]. <br>
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</scene></td></tr>		</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2odu\|2odu]], [[2odv\|2odv]], [[2iak\|2iak]], [[3pe0\|3pe0]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2odu\|2odu]], [[2odv\|2odv]], [[2iak\|2iak]], [[3pe0\|3pe0]]</td></tr>
-	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">PLEC, PLEC1 ([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">PLEC, PLEC1 ([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=3pdy FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3pdy OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3pdy RCSB], [http://www.ebi.ac.uk/pdbsum/3pdy 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=3pdy FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3pdy OCA], [http://pdbe.org/3pdy PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=3pdy RCSB], [http://www.ebi.ac.uk/pdbsum/3pdy PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=3pdy ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 20:		Line 21:
	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 3pdy" style="background-color:#fffaf0;"></div>

	==See Also==		==See Also==
Line 27:		Line 29:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Homo sapiens~~]]	+	[[Category: Human]]
	[[Category: Ortega, E]]		[[Category: Ortega, E]]
	[[Category: Pereda, J M.de]]		[[Category: Pereda, J M.de]]

OCA at 06:51, 19 December 2014

2014-12-19T06:51:23Z

←Older revision		Revision as of 06:51, 19 December 2014
Line 1:		Line 1:
-	~~{{STRUCTURE_3pdy~~\| ~~PDB~~=3pdy \| ~~SCENE~~= }}	+	==Structure of the third and fourth spectrin repeats of the plakin domain of plectin==
-	===~~Structure~~ of the ~~third and fourth~~ spectrin repeats of the plakin domain of plectin~~===~~	+	<StructureSection load='3pdy' size='340' side='right' caption='[[3pdy]], [[Resolution\|resolution]] 2.22Å' scene=''>
-	~~{{ABSTRACT_PUBMED_21288893}}~~	+	== Structural highlights ==
		+	<table><tr><td colspan='2'>[[3pdy]] 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=3PDY OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3PDY FirstGlance]. <br>
		+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</scene></td></tr>
		+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2odu\|2odu]], [[2odv\|2odv]], [[2iak\|2iak]], [[3pe0\|3pe0]]</td></tr>
		+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">PLEC, PLEC1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</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=3pdy FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3pdy OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3pdy RCSB], [http://www.ebi.ac.uk/pdbsum/3pdy PDBsum]</span></td></tr>
		+	</table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Defects in PLEC are the cause of epidermolysis bullosa simplex with pyloric atresia (EBS-PA) [MIM:[http://omim.org/entry/612138 612138]]. EBS-PA is an autosomal recessive genodermatosis characterized by severe skin blistering at birth and congenital pyloric atresia. Death usually occurs in infancy. This disorder is allelic to MD-EBS.<ref>PMID:8698233</ref> <ref>PMID:20665883</ref> <ref>PMID:14675180</ref> Defects in PLEC are the cause of epidermolysis bullosa simplex with muscular dystrophy (MD-EBS) [MIM:[http://omim.org/entry/226670 226670]]. MD-EBS is an autosomal recessive disorder characterized by epidermal blister formation at the level of the hemidesmosome and associated with late-onset muscular dystrophy. Defects in PLEC are the cause of epidermolysis bullosa simplex Ogna type (O-EBS) [MIM:[http://omim.org/entry/131950 131950]]; also called epidermolysis bullosa simplex 1. O-EBS is a form of intraepidermal epidermolysis bullosa characterized by generalized skin bruising, skin fragility with non-scarring blistering and small hemorrhagic blisters on hands. At the ultrastructural level, it is differentiated from classical cases of K-EBS, WC-EBS and DM-EBS, by the occurrence of blisters originating in basal cells above hemidesmosomes, and abnormal hemidesmosome intracellular attachment plates. Defects in PLEC are the cause of limb-girdle muscular dystrophy type 2Q (LGMD2Q) [MIM:[http://omim.org/entry/613723 613723]]. An autosomal recessive degenerative myopathy characterized by early childhood onset of proximal muscle weakness. Note=A 9 bp deletion containing the initiation codon in exon 1f of PLEC have been found in limb-girdle muscular dystrophy patients. The mutation results in deficient expression of isoform 9 and disorganization of the myofibers, without any effect on the skin.<ref>PMID:21109228</ref> <ref>PMID:8698233</ref>
		+	== Function ==
		+	[[http://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, but also in the regulation of their dynamics. Structural component of muscle. Isoform 9 plays a major role in the maintenance of myofibers integrity.<ref>PMID:12482924</ref> <ref>PMID:21109228</ref>
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	Plectin belongs to the plakin family of cytoskeletal crosslinkers, which is part of the spectrin superfamily. Plakins contain an N-terminal conserved region, the plakin domain, which is formed by an array of spectrin repeats (SR) and a Src-homology 3 (SH3), and harbors binding sites for junctional proteins. We have combined x-ray crystallography and small angle x-ray scattering (SAXS) to elucidate the structure of the central region of the plakin domain of plectin, which corresponds to the SR3, SR4, SR5, and SH3 domains. The crystal structures of the SR3-SR4 and SR4-SR5-SH3 fragments were determined to 2.2 and 2.95 A resolution, respectively. The SH3 of plectin presents major alterations as compared with canonical Pro-rich binding SH3 domains, suggesting that plectin does not recognize Pro-rich motifs. In addition, the SH3 binding site is partially occluded by an intramolecular contact with the SR4. Residues of this pseudo-binding site and the SR4/SH3 interface are conserved within the plakin family, suggesting that the structure of this part of the plectin molecule is similar to that of other plakins. We have created a model for the SR3-SR4-SR5-SH3 region, which agrees well with SAXS data in solution. The three SRs form a semi-flexible rod that is not altered by the presence of the SH3 domain, and it is similar to those found in spectrins. The flexibility of the plakin domain, in analogy with spectrins, might contribute to the role of plakins in maintaining the stability of tissues subject to mechanical stress.

-	~~==Disease==~~	+	The Structure of the Plakin Domain of Plectin Reveals a Non-canonical SH3 Domain Interacting with Its Fourth Spectrin Repeat.,Ortega E, Buey RM, Sonnenberg A, de Pereda JM J Biol Chem. 2011 Apr 8;286(14):12429-38. Epub 2011 Feb 1. PMID:21288893<ref>PMID:21288893</ref>
-	~~[[http://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Defects in PLEC are the cause~~ of epidermolysis bullosa simplex with pyloric atresia (EBS-PA) [MIM:[http://omim.org/entry/612138 612138]]. EBS-PA is an autosomal recessive genodermatosis characterized by severe skin blistering at birth and congenital pyloric atresia. Death usually occurs in infancy. This disorder is allelic to MD-EBS.<ref>PMID:8698233</ref><ref>PMID:20665883</ref><ref>PMID:14675180</ref> Defects in PLEC are the ~~cause~~ of ~~epidermolysis bullosa simplex with muscular dystrophy (MD~~-~~EBS) [MIM:[http://omim.org/entry/226670 226670]]. MD-EBS is an autosomal recessive disorder characterized by epidermal blister formation at the level of the hemidesmosome and associated~~ with ~~late-onset muscular dystrophy~~. Defects in PLEC are the cause of epidermolysis bullosa simplex Ogna type (O-EBS) [MIM:[http://omim.org/entry/131950 131950]]; also called epidermolysis bullosa simplex 1. O-EBS is a form of intraepidermal epidermolysis bullosa characterized by generalized skin bruising, ~~skin fragility with non-scarring blistering and small hemorrhagic blisters on hands. At the ultrastructural level~~, ~~it is differentiated from classical cases of K-EBS~~, ~~WC-EBS and DM-EBS~~, ~~by the occurrence of blisters originating in basal cells above hemidesmosomes, and abnormal hemidesmosome intracellular attachment plates~~. ~~Defects in PLEC are the cause of limb-girdle muscular dystrophy type 2Q~~ (~~LGMD2Q~~) ~~[MIM~~:[http://omim.org/entry/613723 613723]]. An autosomal recessive degenerative myopathy characterized by early childhood onset of proximal muscle weakness. Note=A 9 bp deletion containing the initiation codon in exon 1f of PLEC have been found in limb-~~girdle muscular dystrophy patients~~. ~~The mutation results in deficient expression of isoform 9 and disorganization of the myofibers, without any effect on the skin~~.~~<ref>~~PMID:~~21109228</ref>~~<ref>PMID:~~8698233~~</ref>	+

-	~~==Function==~~	+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
-	~~[[http:~~//www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, ~~but also in~~ the ~~regulation of their dynamics~~. ~~Structural component of muscle~~. ~~Isoform 9 plays a major role in the maintenance~~ of ~~myofibers integrity~~.<~~ref~~>~~PMID:12482924~~</~~ref~~>~~<ref>PMID:21109228</ref>~~	+	</div>
-		+
-	~~==About this Structure==~~	+
-	[[3pdy]] 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=3PDY OCA].	+

	==See Also==		==See Also==
	*[[Plectin\|Plectin]]		*[[Plectin\|Plectin]]
-		+	== References ==
-	==~~Reference~~==	+	<references/>
-	~~<ref group="xtra">PMID:021288893</ref>~~<references ~~group="xtra"~~/><~~references~~/>	+	__TOC__
		+	</StructureSection>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
-	[[Category: Ortega, E.]]	+	[[Category: Ortega, E]]
-	[[Category: Pereda, J M.de.]]	+	[[Category: Pereda, J M.de]]
	[[Category: Crosslinking]]		[[Category: Crosslinking]]
	[[Category: Cytoskeleton]]		[[Category: Cytoskeleton]]

OCA at 20:50, 24 March 2013

2013-03-24T20:50:40Z

←Older revision		Revision as of 20:50, 24 March 2013
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-	[[Image:3pdy.png\|left\|200px]]
-
	{{STRUCTURE_3pdy\| PDB=3pdy \| SCENE= }}		{{STRUCTURE_3pdy\| PDB=3pdy \| SCENE= }}
-
	===Structure of the third and fourth spectrin repeats of the plakin domain of plectin===		===Structure of the third and fourth spectrin repeats of the plakin domain of plectin===
		+	{{ABSTRACT_PUBMED_21288893}}

-	~~{{ABSTRACT_PUBMED_21288893}}~~	+	==Disease==
		+	[[http://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Defects in PLEC are the cause of epidermolysis bullosa simplex with pyloric atresia (EBS-PA) [MIM:[http://omim.org/entry/612138 612138]]. EBS-PA is an autosomal recessive genodermatosis characterized by severe skin blistering at birth and congenital pyloric atresia. Death usually occurs in infancy. This disorder is allelic to MD-EBS.<ref>PMID:8698233</ref><ref>PMID:20665883</ref><ref>PMID:14675180</ref> Defects in PLEC are the cause of epidermolysis bullosa simplex with muscular dystrophy (MD-EBS) [MIM:[http://omim.org/entry/226670 226670]]. MD-EBS is an autosomal recessive disorder characterized by epidermal blister formation at the level of the hemidesmosome and associated with late-onset muscular dystrophy. Defects in PLEC are the cause of epidermolysis bullosa simplex Ogna type (O-EBS) [MIM:[http://omim.org/entry/131950 131950]]; also called epidermolysis bullosa simplex 1. O-EBS is a form of intraepidermal epidermolysis bullosa characterized by generalized skin bruising, skin fragility with non-scarring blistering and small hemorrhagic blisters on hands. At the ultrastructural level, it is differentiated from classical cases of K-EBS, WC-EBS and DM-EBS, by the occurrence of blisters originating in basal cells above hemidesmosomes, and abnormal hemidesmosome intracellular attachment plates. Defects in PLEC are the cause of limb-girdle muscular dystrophy type 2Q (LGMD2Q) [MIM:[http://omim.org/entry/613723 613723]]. An autosomal recessive degenerative myopathy characterized by early childhood onset of proximal muscle weakness. Note=A 9 bp deletion containing the initiation codon in exon 1f of PLEC have been found in limb-girdle muscular dystrophy patients. The mutation results in deficient expression of isoform 9 and disorganization of the myofibers, without any effect on the skin.<ref>PMID:21109228</ref><ref>PMID:8698233</ref>
		+
		+	==Function==
		+	[[http://www.uniprot.org/uniprot/PLEC_HUMAN PLEC_HUMAN]] Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, but also in the regulation of their dynamics. Structural component of muscle. Isoform 9 plays a major role in the maintenance of myofibers integrity.<ref>PMID:12482924</ref><ref>PMID:21109228</ref>

	==About this Structure==		==About this Structure==
Line 14:		Line 16:

	==Reference==		==Reference==
-	<ref group="xtra">PMID:021288893</ref><references group="xtra"/>	+	<ref group="xtra">PMID:021288893</ref><references group="xtra"/><references/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Ortega, E.]]		[[Category: Ortega, E.]]

OCA at 11:18, 20 October 2012

2012-10-20T11:18:35Z

←Older revision		Revision as of 11:18, 20 October 2012
Line 8:		Line 8:

	==About this Structure==		==About this Structure==
-	[[3pdy]] is a 2 chain structure ~~of [[Plectin]]~~ 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=3PDY OCA].	+	[[3pdy]] 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=3PDY OCA].

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

OCA at 04:55, 26 July 2012

2012-07-26T04:55:08Z

←Older revision		Revision as of 04:55, 26 July 2012
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	[[Image:3pdy.png\|left\|200px]]		[[Image:3pdy.png\|left\|200px]]

-	<!--
-	The line below this paragraph, containing "STRUCTURE_3pdy", 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_3pdy\| PDB=3pdy \| SCENE= }}		{{STRUCTURE_3pdy\| PDB=3pdy \| SCENE= }}

	===Structure of the third and fourth spectrin repeats of the plakin domain of plectin===		===Structure of the third and fourth spectrin repeats of the plakin domain of plectin===

-
-	<!--
-	The line below this paragraph, {{ABSTRACT_PUBMED_21288893}}, adds the Publication Abstract to the page
-	(as it appears on PubMed at http://www.pubmed.gov), where 21288893 is the PubMed ID number.
-	-->
	{{ABSTRACT_PUBMED_21288893}}		{{ABSTRACT_PUBMED_21288893}}

	==About this Structure==		==About this Structure==
-	[[3pdy]] 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=3PDY OCA].	+	[[3pdy]] is a 2 chain structure of [[Plectin]] 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=3PDY OCA].
		+
		+	==See Also==
		+	*[[Plectin\|Plectin]]

	==Reference==		==Reference==
-	<ref group="xtra">PMID:~~21288893~~</ref><references group="xtra"/>	+	<ref group="xtra">PMID:021288893</ref><references group="xtra"/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Ortega, E.]]		[[Category: Ortega, E.]]
	[[Category: Pereda, J M.de.]]		[[Category: Pereda, J M.de.]]
		+	[[Category: Crosslinking]]
		+	[[Category: Cytoskeleton]]
		+	[[Category: Intermediate filament]]
		+	[[Category: Plakin]]
		+	[[Category: Spectrin repeat]]
		+	[[Category: Structural protein]]

OCA at 09:56, 21 April 2011

2011-04-21T09:56:22Z

←Older revision		Revision as of 09:56, 21 April 2011
Line 1:		Line 1:
-	[[Image:3pdy.~~jpg~~\|left\|200px]]	+	[[Image:3pdy.png\|left\|200px]]

	<!--		<!--
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	===Structure of the third and fourth spectrin repeats of the plakin domain of plectin===		===Structure of the third and fourth spectrin repeats of the plakin domain of plectin===

		+
		+	<!--
		+	The line below this paragraph, {{ABSTRACT_PUBMED_21288893}}, adds the Publication Abstract to the page
		+	(as it appears on PubMed at http://www.pubmed.gov), where 21288893 is the PubMed ID number.
		+	-->
		+	{{ABSTRACT_PUBMED_21288893}}

	==About this Structure==		==About this Structure==
	[[3pdy]] 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=3PDY OCA].		[[3pdy]] 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=3PDY OCA].
		+
		+	==Reference==
		+	<ref group="xtra">PMID:21288893</ref><references group="xtra"/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Ortega, E.]]		[[Category: Ortega, E.]]
	[[Category: Pereda, J M.de.]]		[[Category: Pereda, J M.de.]]

OCA: Protected "3pdy" [edit=sysop:move=sysop]

2011-02-02T06:32:19Z

Protected "3pdy" [edit=sysop:move=sysop]

←Older revision

Revision as of 06:32, 2 February 2011

OCA at 06:32, 2 February 2011

2011-02-02T06:32:16Z

←Older revision		Revision as of 06:32, 2 February 2011
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-	~~'''Unreleased structure'''~~	+	[[Image:3pdy.jpg\|left\|200px]]

-	The ~~entry 3pdy~~ is ~~ON HOLD~~ ~~until Paper Publication~~	+	<!--
		+	The line below this paragraph, containing "STRUCTURE_3pdy", 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_3pdy\| PDB=3pdy \| SCENE= }}

-	~~Authors: Ortega, E., de Pereda, J.M.~~	+	===Structure of the third and fourth spectrin repeats of the plakin domain of plectin===

-	Description: Structure of the third and fourth spectrin repeats of the plakin domain of plectin

-	~~''Page seeded by~~ [http://oca.weizmann.ac.il/oca OCA ] ~~on Wed Nov 10 06~~:32:~~33 2010''~~	+	==About this Structure==
		+	[[3pdy]] 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=3PDY OCA].
		+	[[Category: Homo sapiens]]
		+	[[Category: Ortega, E.]]
		+	[[Category: Pereda, J M.de.]]