6jco - Revision history

OCA at 10:06, 22 November 2023

OCA — Wed, 22 Nov 2023 10:06:39 GMT

←Older revision		Revision as of 10:06, 22 November 2023
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	<StructureSection load='6jco' size='340' side='right'caption='[[6jco]], [[Resolution\|resolution]] 2.88Å' scene=''>		<StructureSection load='6jco' size='340' side='right'caption='[[6jco]], [[Resolution\|resolution]] 2.88Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[6jco]] 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=6JCO OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=6JCO FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[6jco]] 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=6JCO OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6JCO FirstGlance]. <br>
-	</td></tr><tr id='~~gene~~'><td class="sblockLbl"><b>[[~~Gene~~\|~~Gene~~:]]</b></td><td class="sblockDat">~~GSN (~~[~~http://www~~.~~ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info~~&~~srchmode=5&id=9606 HUMAN])~~</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]] 2.88Å</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=6jco FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6jco OCA], [~~http~~://pdbe.org/6jco PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=6jco RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/6jco PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=6jco 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=6jco FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6jco OCA], [https://pdbe.org/6jco PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6jco RCSB], [https://www.ebi.ac.uk/pdbsum/6jco PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6jco ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
-	[~~[http~~://www.uniprot.org/uniprot/GELS_HUMAN GELS_HUMAN]] Defects in GSN are the cause of amyloidosis type 5 (AMYL5) [MIM:[~~http~~://omim.org/entry/105120 105120]]; also known as familial amyloidosis Finnish type. AMYL5 is a hereditary generalized amyloidosis due to gelsolin amyloid deposition. It is typically characterized by cranial neuropathy and lattice corneal dystrophy. Most patients have modest involvement of internal organs, but severe systemic disease can develop in some individuals causing peripheral polyneuropathy, amyloid cardiomyopathy, and nephrotic syndrome leading to renal failure.<ref>PMID:2157434</ref> <ref>PMID:2153578</ref> <ref>PMID:2176481</ref> <ref>PMID:1338910</ref>	+	[https://www.uniprot.org/uniprot/GELS_HUMAN GELS_HUMAN] Defects in GSN are the cause of amyloidosis type 5 (AMYL5) [MIM:[https://omim.org/entry/105120 105120]; also known as familial amyloidosis Finnish type. AMYL5 is a hereditary generalized amyloidosis due to gelsolin amyloid deposition. It is typically characterized by cranial neuropathy and lattice corneal dystrophy. Most patients have modest involvement of internal organs, but severe systemic disease can develop in some individuals causing peripheral polyneuropathy, amyloid cardiomyopathy, and nephrotic syndrome leading to renal failure.<ref>PMID:2157434</ref> <ref>PMID:2153578</ref> <ref>PMID:2176481</ref> <ref>PMID:1338910</ref>
	== Function ==		== Function ==
-	[~~[http~~://www.uniprot.org/uniprot/GELS_HUMAN GELS_HUMAN]] Calcium-regulated, actin-modulating protein that binds to the plus (or barbed) ends of actin monomers or filaments, preventing monomer exchange (end-blocking or capping). It can promote the assembly of monomers into filaments (nucleation) as well as sever filaments already formed. Plays a role in ciliogenesis.<ref>PMID:20393563</ref>	+	[https://www.uniprot.org/uniprot/GELS_HUMAN GELS_HUMAN] Calcium-regulated, actin-modulating protein that binds to the plus (or barbed) ends of actin monomers or filaments, preventing monomer exchange (end-blocking or capping). It can promote the assembly of monomers into filaments (nucleation) as well as sever filaments already formed. Plays a role in ciliogenesis.<ref>PMID:20393563</ref>
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
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	==See Also==		==See Also==
-	*[[3D ~~Structures of gelsolin~~\|3D ~~Structures of gelsolin~~]]	+	*[[Gelsolin 3D structures\|Gelsolin 3D structures]]
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Human~~]]	+	[[Category: Homo sapiens]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	[[Category: Robinson~~, R C~~]]	+	[[Category: Robinson RC]]
-	[[Category: Zorgati, H]]	+	[[Category: Zorgati H]]
-	~~[[Category: Actin binding protein]]~~	+
-	~~[[Category: Amyloidosis]]~~	+
-	~~[[Category: Calcium activation]]~~	+
-	~~[[Category: Gelsolin~~]]	+

OCA at 08:37, 24 July 2019

OCA — Wed, 24 Jul 2019 08:37:13 GMT

←Older revision		Revision as of 08:37, 24 July 2019
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	<StructureSection load='6jco' size='340' side='right'caption='[[6jco]], [[Resolution\|resolution]] 2.88Å' scene=''>		<StructureSection load='6jco' size='340' side='right'caption='[[6jco]], [[Resolution\|resolution]] 2.88Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[6jco]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6JCO OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6JCO FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[6jco]] 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=6JCO OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6JCO FirstGlance]. <br>
-	</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=6jco FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6jco OCA], [http://pdbe.org/6jco PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6jco RCSB], [http://www.ebi.ac.uk/pdbsum/6jco PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6jco ProSAT]</span></td></tr>	+	</td></tr><tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">GSN ([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=6jco FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6jco OCA], [http://pdbe.org/6jco PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6jco RCSB], [http://www.ebi.ac.uk/pdbsum/6jco PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6jco ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 10:		Line 11:
	== Function ==		== Function ==
	[[http://www.uniprot.org/uniprot/GELS_HUMAN GELS_HUMAN]] Calcium-regulated, actin-modulating protein that binds to the plus (or barbed) ends of actin monomers or filaments, preventing monomer exchange (end-blocking or capping). It can promote the assembly of monomers into filaments (nucleation) as well as sever filaments already formed. Plays a role in ciliogenesis.<ref>PMID:20393563</ref>		[[http://www.uniprot.org/uniprot/GELS_HUMAN GELS_HUMAN]] Calcium-regulated, actin-modulating protein that binds to the plus (or barbed) ends of actin monomers or filaments, preventing monomer exchange (end-blocking or capping). It can promote the assembly of monomers into filaments (nucleation) as well as sever filaments already formed. Plays a role in ciliogenesis.<ref>PMID:20393563</ref>
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	In the disease familial amyloidosis, Finnish type (FAF), also known as AGel amyloidosis (AGel), the mechanism by which point mutations in the calcium-regulated actin-severing protein gelsolin lead to furin cleavage is not understood in the intact protein. Here, we provide a structural and biochemical characterization of the FAF variants. X-ray crystallography structures of the FAF mutant gelsolins demonstrate that the mutations do not significantly disrupt the calcium-free conformations of gelsolin. Small-angle X-ray-scattering (SAXS) studies indicate that the FAF calcium-binding site mutants are slower to activate, whereas G167R is as efficient as the wild type. Actin-regulating studies of the gelsolins at the furin cleavage pH (6.5) show that the mutant gelsolins are functional, suggesting that they also adopt relatively normal active conformations. Deletion of gelsolin domains leads to sensitization to furin cleavage, and nanobody-binding protects against furin cleavage. These data indicate instability in the second domain of gelsolin (G2), since loss or gain of G2-stabilizing interactions impacts the efficiency of cleavage by furin. To demonstrate this principle, we engineered non-FAF mutations in G3 that disrupt the G2-G3 interface in the calcium-activated structure. These mutants led to increased furin cleavage. We carried out molecular dynamics (MD) simulations on the FAF and non-FAF mutant G2-G3 fragments of gelsolin. All mutants showed an increase in the distance between the center of masses of the 2 domains (G2 and G3). Since G3 covers the furin cleavage site on G2 in calcium-activated gelsolin, this suggests that destabilization of this interface is a critical step in cleavage.
		+
		+	The role of gelsolin domain 3 in familial amyloidosis (Finnish type).,Zorgati H, Larsson M, Ren W, Sim AYL, Gettemans J, Grimes JM, Li W, Robinson RC Proc Natl Acad Sci U S A. 2019 Jul 9;116(28):13958-13963. doi:, 10.1073/pnas.1902189116. Epub 2019 Jun 26. PMID:31243148<ref>PMID:31243148</ref>
		+
		+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
		+	</div>
		+	<div class="pdbe-citations 6jco" style="background-color:#fffaf0;"></div>
		+
		+	==See Also==
		+	*[[3D Structures of gelsolin\|3D Structures of gelsolin]]
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
		+	[[Category: Human]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
	[[Category: Robinson, R C]]		[[Category: Robinson, R C]]

OCA at 05:35, 12 June 2019

OCA — Wed, 12 Jun 2019 05:35:59 GMT

←Older revision		Revision as of 05:35, 12 June 2019
Line 1:		Line 1:
-	'''Unreleased structure'''

-	~~The entry~~ 6jco is ~~ON HOLD until Paper Publication~~	+	==Crystal structure of calcium free human gelsolin amyloid mutant D187N==
-		+	<StructureSection load='6jco' size='340' side='right'caption='[[6jco]], [[Resolution\|resolution]] 2.88Å' scene=''>
-	~~Authors~~:	+	== Structural highlights ==
-		+	<table><tr><td colspan='2'>[[6jco]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6JCO OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6JCO FirstGlance]. <br>
-	~~Description~~:	+	</td></tr><tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6jco FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6jco OCA], [http://pdbe.org/6jco PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6jco RCSB], [http://www.ebi.ac.uk/pdbsum/6jco PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6jco ProSAT]</span></td></tr>
-	[[Category: ~~Unreleased~~ Structures]]	+	</table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/GELS_HUMAN GELS_HUMAN]] Defects in GSN are the cause of amyloidosis type 5 (AMYL5) [MIM:[http://omim.org/entry/105120 105120]]; also known as familial amyloidosis Finnish type. AMYL5 is a hereditary generalized amyloidosis due to gelsolin amyloid deposition. It is typically characterized by cranial neuropathy and lattice corneal dystrophy. Most patients have modest involvement of internal organs, but severe systemic disease can develop in some individuals causing peripheral polyneuropathy, amyloid cardiomyopathy, and nephrotic syndrome leading to renal failure.<ref>PMID:2157434</ref> <ref>PMID:2153578</ref> <ref>PMID:2176481</ref> <ref>PMID:1338910</ref>
		+	== Function ==
		+	[[http://www.uniprot.org/uniprot/GELS_HUMAN GELS_HUMAN]] Calcium-regulated, actin-modulating protein that binds to the plus (or barbed) ends of actin monomers or filaments, preventing monomer exchange (end-blocking or capping). It can promote the assembly of monomers into filaments (nucleation) as well as sever filaments already formed. Plays a role in ciliogenesis.<ref>PMID:20393563</ref>
		+	== References ==
		+	<references/>
		+	__TOC__
		+	</StructureSection>
		+	[[Category: Large Structures]]
		+	[[Category: Robinson, R C]]
		+	[[Category: Zorgati, H]]
		+	[[Category: Actin binding protein]]
		+	[[Category: Amyloidosis]]
		+	[[Category: Calcium activation]]
		+	[[Category: Gelsolin]]

OCA at 06:52, 1 May 2019

OCA — Wed, 01 May 2019 06:52:07 GMT

←Older revision		Revision as of 06:52, 1 May 2019
Line 1:		Line 1:
	'''Unreleased structure'''		'''Unreleased structure'''

-	The entry 6jco is ON HOLD	+	The entry 6jco is ON HOLD until Paper Publication

	Authors:		Authors:

OCA: Protected "6jco" [edit=sysop:move=sysop]

OCA — Wed, 13 Feb 2019 07:23:00 GMT

Protected "6jco" [edit=sysop:move=sysop]

←Older revision

Revision as of 07:23, 13 February 2019

OCA: New page: '''Unreleased structure''' The entry 6jco is ON HOLD Authors: Description: Category: Unreleased Structures

OCA — Wed, 13 Feb 2019 07:22:58 GMT

New page: '''Unreleased structure''' The entry 6jco is ON HOLD Authors: Description: Category: Unreleased Structures

New page

'''Unreleased structure'''

The entry 6jco is ON HOLD

Authors:

Description:
[[Category: Unreleased Structures]]