5v44 - Revision history

OCA at 13:41, 4 October 2023

2023-10-04T13:41:17Z

←Older revision		Revision as of 13:41, 4 October 2023
Line 1:		Line 1:

	==Crystal structure of the SR1 domain of human sacsin==		==Crystal structure of the SR1 domain of human sacsin==
-	<StructureSection load='5v44' size='340' side='right' caption='[[5v44]], [[Resolution\|resolution]] 1.56Å' scene=''>	+	<StructureSection load='5v44' size='340' side='right'caption='[[5v44]], [[Resolution\|resolution]] 1.56Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[5v44]] is a 3 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=5V44 OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=5V44 FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[5v44]] is a 3 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=5V44 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=5V44 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~~></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]] 1.56Å</td></tr>
-	<tr id='~~NonStdRes~~'><td class="sblockLbl"><b>[[~~Non-Standard_Residue~~\|~~NonStd Res~~:]]</b></td><td class="sblockDat"><scene name='pdbligand=~~MSE~~:~~SELENOMETHIONINE~~'>~~MSE~~</scene><~~/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=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
-	~~<tr id~~='~~related'><td class~~=~~"sblockLbl"><b>[[Related_structure\|Related~~:~~]]</b></td><td class="sblockDat">[[5v45\|5v45]], [[5v46\|5v46]], [[5v47\|5v47]]</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=5v44 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5v44 OCA], [https://pdbe.org/5v44 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=5v44 RCSB], [https://www.ebi.ac.uk/pdbsum/5v44 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=5v44 ProSAT]</span></td></tr>
-	~~<tr id='gene~~'>~~<td class="sblockLbl"><b>[[Gene\|Gene:]]~~</b>~~</td><td class="sblockDat">SACS, KIAA0730 ([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=5v44 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5v44 OCA], [~~http~~://pdbe.org/5v44 PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=5v44 RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/5v44 PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=5v44 ProSAT]</span></td></tr>	+
	</table>		</table>
	== Disease ==		== Disease ==
-	[~~[http~~://www.uniprot.org/uniprot/SACS_HUMAN SACS_HUMAN]] Defects in SACS are the cause of spastic ataxia Charlevoix-Saguenay type (SACS) [MIM:[~~http~~://omim.org/entry/270550 270550]]. It is a neurodegenerative disease characterized by early-onset cerebellar ataxia, spasticity, retinal hypermyelination, pyramidal signs, and both axonal and demyelinating neuropathy with loss of sensory nerve conduction and reduced motor conduction velocities. Other features include dysarthria, distal muscle wasting, nystagmus, defect in conjugate pursuit ocular movements, retinal striation (from prominent retinal nerves) obscuring the retinal blood vessels in places, and the frequent presence of mitral valve prolapse.<ref>PMID:10655055</ref> <ref>PMID:19529988</ref> <ref>PMID:12873855</ref> <ref>PMID:15156359</ref> <ref>PMID:14718708</ref> <ref>PMID:16007637</ref> <ref>PMID:15985586</ref> <ref>PMID:17290461</ref> <ref>PMID:18398442</ref> <ref>PMID:18484239</ref> <ref>PMID:17716690</ref> <ref>PMID:18465152</ref> <ref>PMID:20876471</ref>	+	[https://www.uniprot.org/uniprot/SACS_HUMAN SACS_HUMAN] Defects in SACS are the cause of spastic ataxia Charlevoix-Saguenay type (SACS) [MIM:[https://omim.org/entry/270550 270550]. It is a neurodegenerative disease characterized by early-onset cerebellar ataxia, spasticity, retinal hypermyelination, pyramidal signs, and both axonal and demyelinating neuropathy with loss of sensory nerve conduction and reduced motor conduction velocities. Other features include dysarthria, distal muscle wasting, nystagmus, defect in conjugate pursuit ocular movements, retinal striation (from prominent retinal nerves) obscuring the retinal blood vessels in places, and the frequent presence of mitral valve prolapse.<ref>PMID:10655055</ref> <ref>PMID:19529988</ref> <ref>PMID:12873855</ref> <ref>PMID:15156359</ref> <ref>PMID:14718708</ref> <ref>PMID:16007637</ref> <ref>PMID:15985586</ref> <ref>PMID:17290461</ref> <ref>PMID:18398442</ref> <ref>PMID:18484239</ref> <ref>PMID:17716690</ref> <ref>PMID:18465152</ref> <ref>PMID:20876471</ref>
	== Function ==		== Function ==
-	[~~[http~~://www.uniprot.org/uniprot/SACS_HUMAN SACS_HUMAN]] Co-chaperone which acts as a regulator of the Hsp70 chaperone machinery and may be involved in the processing of other ataxia-linked proteins.<ref>PMID:19208651</ref>	+	[https://www.uniprot.org/uniprot/SACS_HUMAN SACS_HUMAN] Co-chaperone which acts as a regulator of the Hsp70 chaperone machinery and may be involved in the processing of other ataxia-linked proteins.<ref>PMID:19208651</ref>
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
Line 23:		Line 21:
	</div>		</div>
	<div class="pdbe-citations 5v44" style="background-color:#fffaf0;"></div>		<div class="pdbe-citations 5v44" style="background-color:#fffaf0;"></div>
		+
		+	==See Also==
		+	*[[DnaJ homolog 3D structures\|DnaJ homolog 3D structures]]
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Human~~]]	+	[[Category: Homo sapiens]]
-	[[Category: ~~Gehring, K~~]]	+	[[Category: Large Structures]]
-	[[Category: ~~Kozlov, G~~]]	+	[[Category: Gehring K]]
-	[[Category: ~~Menade, M~~]]	+	[[Category: Kozlov G]]
-	[[Category: ~~Alpha-beta sandwich]]~~	+	[[Category: Menade M]]
-	~~[[Category: Bergerat fold]]~~	+
-	~~[[Category: Chaperone~~]]	+

OCA at 06:07, 11 July 2018

2018-07-11T06:07:10Z

←Older revision		Revision as of 06:07, 11 July 2018
Line 3:		Line 3:
	<StructureSection load='5v44' size='340' side='right' caption='[[5v44]], [[Resolution\|resolution]] 1.56Å' scene=''>		<StructureSection load='5v44' size='340' side='right' caption='[[5v44]], [[Resolution\|resolution]] 1.56Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[5v44]] is a 3 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5V44 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5V44 FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[5v44]] is a 3 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=5V44 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5V44 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></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></td></tr>
	<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue\|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>		<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue\|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[5v45\|5v45]], [[5v46\|5v46]], [[5v47\|5v47]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[5v45\|5v45]], [[5v46\|5v46]], [[5v47\|5v47]]</td></tr>
		+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">SACS, KIAA0730 ([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=5v44 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5v44 OCA], [http://pdbe.org/5v44 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5v44 RCSB], [http://www.ebi.ac.uk/pdbsum/5v44 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5v44 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=5v44 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5v44 OCA], [http://pdbe.org/5v44 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5v44 RCSB], [http://www.ebi.ac.uk/pdbsum/5v44 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5v44 ProSAT]</span></td></tr>
	</table>		</table>
Line 13:		Line 14:
	== Function ==		== Function ==
	[[http://www.uniprot.org/uniprot/SACS_HUMAN SACS_HUMAN]] Co-chaperone which acts as a regulator of the Hsp70 chaperone machinery and may be involved in the processing of other ataxia-linked proteins.<ref>PMID:19208651</ref>		[[http://www.uniprot.org/uniprot/SACS_HUMAN SACS_HUMAN]] Co-chaperone which acts as a regulator of the Hsp70 chaperone machinery and may be involved in the processing of other ataxia-linked proteins.<ref>PMID:19208651</ref>
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a neurodegenerative disease that is caused by mutations in the SACS gene. The product of this gene is a very large 520 kDa cytoplasmic protein, sacsin, with an ubiquitin-like domain (Ubl) at the N-terminus followed by three large sacsin internal repeats (SIRPTs) supradomains and C-terminal J and HEPN domains. The SIRPTs are predicted to contain Hsp90-like domains suggesting a potential chaperone activity. In this work, we report the structures of the Hsp90-like Sr1 domain of SIRPT1 and the N-terminal Ubl domain determined at 1.55 A and 2.1 A resolution, respectively. The Ubl domain crystallized as a swapped dimer that could be relevant in the context of full-length protein. The Sr1 domain displays the Bergerat protein fold with a characteristic nucleotide-binding pocket, though it binds nucleotides with very low affinity. The Sr1 structure reveals that ARSACS-causing missense mutations (R272H, R272C and T201K) disrupt protein folding most likely leading to sacsin degradation. This work lends a structural support to the view of sacsin as a molecular chaperone and provides a framework for future studies of this protein.
		+
		+	Structures of Ubl and Hsp90-like domains of sacsin provide insight into pathological mutations.,Menade M, Kozlov G, Trempe JF, Pande H, Shenker S, Wickremasinghe S, Li X, Hojjat H, Dicaire MJ, Brais B, McPherson PS, Wong MJH, Young JC, Gehring K J Biol Chem. 2018 Jun 26. pii: RA118.003939. doi: 10.1074/jbc.RA118.003939. PMID:29945973<ref>PMID:29945973</ref>
		+
		+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
		+	</div>
		+	<div class="pdbe-citations 5v44" style="background-color:#fffaf0;"></div>
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
		+	[[Category: Human]]
	[[Category: Gehring, K]]		[[Category: Gehring, K]]
	[[Category: Kozlov, G]]		[[Category: Kozlov, G]]

OCA at 13:50, 24 May 2017

2017-05-24T13:50:56Z

←Older revision		Revision as of 13:50, 24 May 2017
Line 1:		Line 1:
-	'''Unreleased structure'''

-	~~The entry~~ 5v44 is ~~ON HOLD until Paper Publication~~	+	==Crystal structure of the SR1 domain of human sacsin==
-		+	<StructureSection load='5v44' size='340' side='right' caption='[[5v44]], [[Resolution\|resolution]] 1.56Å' scene=''>
-	~~Authors~~: ~~Menade~~, M., ~~Kozlov~~, G., ~~Gehring~~, K.	+	== Structural highlights ==
-		+	<table><tr><td colspan='2'>[[5v44]] is a 3 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5V44 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5V44 FirstGlance]. <br>
-	~~Description~~: ~~Crystal structure~~ of the ~~SR1 domain~~ of ~~human sacsin~~	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></td></tr>
-	[[Category: ~~Unreleased Structures~~]]	+	<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue\|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
		+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[5v45\|5v45]], [[5v46\|5v46]], [[5v47\|5v47]]</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=5v44 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5v44 OCA], [http://pdbe.org/5v44 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5v44 RCSB], [http://www.ebi.ac.uk/pdbsum/5v44 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5v44 ProSAT]</span></td></tr>
		+	</table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/SACS_HUMAN SACS_HUMAN]] Defects in SACS are the cause of spastic ataxia Charlevoix-Saguenay type (SACS) [MIM:[http://omim.org/entry/270550 270550]]. It is a neurodegenerative disease characterized by early-onset cerebellar ataxia, spasticity, retinal hypermyelination, pyramidal signs, and both axonal and demyelinating neuropathy with loss of sensory nerve conduction and reduced motor conduction velocities. Other features include dysarthria, distal muscle wasting, nystagmus, defect in conjugate pursuit ocular movements, retinal striation (from prominent retinal nerves) obscuring the retinal blood vessels in places, and the frequent presence of mitral valve prolapse.<ref>PMID:10655055</ref> <ref>PMID:19529988</ref> <ref>PMID:12873855</ref> <ref>PMID:15156359</ref> <ref>PMID:14718708</ref> <ref>PMID:16007637</ref> <ref>PMID:15985586</ref> <ref>PMID:17290461</ref> <ref>PMID:18398442</ref> <ref>PMID:18484239</ref> <ref>PMID:17716690</ref> <ref>PMID:18465152</ref> <ref>PMID:20876471</ref>
		+	== Function ==
		+	[[http://www.uniprot.org/uniprot/SACS_HUMAN SACS_HUMAN]] Co-chaperone which acts as a regulator of the Hsp70 chaperone machinery and may be involved in the processing of other ataxia-linked proteins.<ref>PMID:19208651</ref>
		+	== References ==
		+	<references/>
		+	__TOC__
		+	</StructureSection>
		+	[[Category: Gehring, K]]
		+	[[Category: Kozlov, G]]
	[[Category: Menade, M]]		[[Category: Menade, M]]
-	[[Category: ~~Kozlov, G~~]]	+	[[Category: Alpha-beta sandwich]]
-	[[Category: ~~Gehring, K~~]]	+	[[Category: Bergerat fold]]
		+	[[Category: Chaperone]]

OCA at 11:34, 5 April 2017

2017-04-05T11:34:28Z

←Older revision		Revision as of 11:34, 5 April 2017
Line 1:		Line 1:
	'''Unreleased structure'''		'''Unreleased structure'''

-	The entry 5v44 is ON HOLD	+	The entry 5v44 is ON HOLD until Paper Publication

	Authors: Menade, M., Kozlov, G., Gehring, K.		Authors: Menade, M., Kozlov, G., Gehring, K.

OCA: Protected "5v44" [edit=sysop:move=sysop]

2017-03-15T19:44:35Z

Protected "5v44" [edit=sysop:move=sysop]

←Older revision

Revision as of 19:44, 15 March 2017

OCA: New page: '''Unreleased structure''' The entry 5v44 is ON HOLD Authors: Menade, M., Kozlov, G., Gehring, K. Description: Crystal structure of the SR1 domain of human sacsin [[Category: Unrelease...

2017-03-15T19:44:33Z

New page: '''Unreleased structure''' The entry 5v44 is ON HOLD Authors: Menade, M., Kozlov, G., Gehring, K. Description: Crystal structure of the SR1 domain of human sacsin [[Category: Unrelease...

New page

'''Unreleased structure'''

The entry 5v44 is ON HOLD

Authors: Menade, M., Kozlov, G., Gehring, K.

Description: Crystal structure of the SR1 domain of human sacsin
[[Category: Unreleased Structures]]
[[Category: Menade, M]]
[[Category: Kozlov, G]]
[[Category: Gehring, K]]