4nxs - Revision history

OCA at 17:04, 20 September 2023

2023-09-20T17:04:39Z

←Older revision		Revision as of 17:04, 20 September 2023
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	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[4nxs]] 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=4NXS OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4NXS FirstGlance]. <br>		<table><tr><td colspan='2'>[[4nxs]] 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=4NXS OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4NXS FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</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]] 2.5493Å</td></tr>
		+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></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=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [https://pdbe.org/4nxs PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [https://www.ebi.ac.uk/pdbsum/4nxs PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4nxs 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=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [https://pdbe.org/4nxs PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [https://www.ebi.ac.uk/pdbsum/4nxs PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4nxs ProSAT]</span></td></tr>
	</table>		</table>

OCA at 08:44, 18 January 2023

2023-01-18T08:44:51Z

←Older revision		Revision as of 08:44, 18 January 2023
Line 1:		Line 1:

	==Crystal structure of human alpha-galactosidase A in complex with 1-deoxygalactonojirimycin-pFPhT==		==Crystal structure of human alpha-galactosidase A in complex with 1-deoxygalactonojirimycin-pFPhT==
-	<StructureSection load='4nxs' size='340' side='right' caption='[[4nxs]], [[Resolution\|resolution]] 2.55Å' scene=''>	+	<StructureSection load='4nxs' size='340' side='right'caption='[[4nxs]], [[Resolution\|resolution]] 2.55Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4nxs]] 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=4NXS OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=4NXS FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4nxs]] 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=4NXS OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4NXS FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</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=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
-	~~<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">GLA ([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'>[https://proteopedia.org/fgij/fg.htm?mol=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [https://pdbe.org/4nxs PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [https://www.ebi.ac.uk/pdbsum/4nxs PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4nxs ProSAT]</span></td></tr>
-	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Alpha-galactosidase Alpha-galactosidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.22 3.2.1.22] </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=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [~~http~~://pdbe.org/4nxs PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/4nxs PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=4nxs ProSAT]</span></td></tr>	+
	</table>		</table>
	== Disease ==		== Disease ==
-	[~~[http~~://www.uniprot.org/uniprot/AGAL_HUMAN AGAL_HUMAN]] Defects in GLA are the cause of Fabry disease (FD) [MIM:[~~http~~://omim.org/entry/301500 301500]]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.<ref>PMID:2152885</ref> <ref>PMID:1846223</ref> <ref>PMID:2171331</ref> <ref>PMID:2539398</ref> <ref>PMID:1315715</ref> <ref>PMID:7504405</ref> <ref>PMID:8395937</ref> <ref>PMID:8069316</ref> <ref>PMID:7531540</ref> <ref>PMID:7575533</ref> <ref>PMID:7759078</ref> <ref>PMID:7599642</ref> <ref>PMID:7596372</ref> <ref>PMID:8738659</ref> <ref>PMID:8875188</ref> <ref>PMID:8834244</ref> <ref>PMID:8931708</ref> <ref>PMID:8807334</ref> <ref>PMID:8863162</ref> <ref>PMID:9105656</ref> <ref>PMID:9100224</ref> <ref>PMID:9554750</ref> <ref>PMID:9452068</ref> <ref>PMID:9452090</ref> <ref>PMID:9452111</ref> <ref>PMID:10208848</ref> <ref>PMID:10090526</ref> <ref>PMID:10838196</ref> <ref>PMID:10666480</ref> <ref>PMID:11076046</ref> <ref>PMID:10916280</ref> <ref>PMID:11295840</ref> <ref>PMID:11668641</ref> <ref>PMID:11889412</ref> <ref>PMID:12694230</ref> <ref>PMID:12786754</ref> <ref>PMID:15162124</ref> <ref>PMID:15712228</ref> <ref>PMID:16533976</ref> <ref>PMID:19621417</ref>	+	[https://www.uniprot.org/uniprot/AGAL_HUMAN AGAL_HUMAN] Defects in GLA are the cause of Fabry disease (FD) [MIM:[https://omim.org/entry/301500 301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.<ref>PMID:2152885</ref> <ref>PMID:1846223</ref> <ref>PMID:2171331</ref> <ref>PMID:2539398</ref> <ref>PMID:1315715</ref> <ref>PMID:7504405</ref> <ref>PMID:8395937</ref> <ref>PMID:8069316</ref> <ref>PMID:7531540</ref> <ref>PMID:7575533</ref> <ref>PMID:7759078</ref> <ref>PMID:7599642</ref> <ref>PMID:7596372</ref> <ref>PMID:8738659</ref> <ref>PMID:8875188</ref> <ref>PMID:8834244</ref> <ref>PMID:8931708</ref> <ref>PMID:8807334</ref> <ref>PMID:8863162</ref> <ref>PMID:9105656</ref> <ref>PMID:9100224</ref> <ref>PMID:9554750</ref> <ref>PMID:9452068</ref> <ref>PMID:9452090</ref> <ref>PMID:9452111</ref> <ref>PMID:10208848</ref> <ref>PMID:10090526</ref> <ref>PMID:10838196</ref> <ref>PMID:10666480</ref> <ref>PMID:11076046</ref> <ref>PMID:10916280</ref> <ref>PMID:11295840</ref> <ref>PMID:11668641</ref> <ref>PMID:11889412</ref> <ref>PMID:12694230</ref> <ref>PMID:12786754</ref> <ref>PMID:15162124</ref> <ref>PMID:15712228</ref> <ref>PMID:16533976</ref> <ref>PMID:19621417</ref>
		+	== Function ==
		+	[https://www.uniprot.org/uniprot/AGAL_HUMAN AGAL_HUMAN]
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
Line 20:		Line 20:
	</div>		</div>
	<div class="pdbe-citations 4nxs" style="background-color:#fffaf0;"></div>		<div class="pdbe-citations 4nxs" style="background-color:#fffaf0;"></div>
		+
		+	==See Also==
		+	*[[Galactosidase 3D structures\|Galactosidase 3D structures]]
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Alpha-galactosidase~~]]	+	[[Category: Homo sapiens]]
-	[[Category: ~~Human~~]]	+	[[Category: Large Structures]]
-	[[Category: Drury~~, J E~~]]	+	[[Category: Drury JE]]
-	[[Category: Johnson~~, J L~~]]	+	[[Category: Johnson JL]]
-	[[Category: Lieberman~~, R L]]~~	+	[[Category: Lieberman RL]]
-	~~[[Category: Arylthiourea]]~~	+
-	~~[[Category: Drug delivery system]]~~	+
-	~~[[Category: Enzyme activation]]~~	+
-	~~[[Category: Enzyme stability]]~~	+
-	~~[[Category: Fabry disease]]~~	+
-	~~[[Category: Gla gene]]~~	+
-	~~[[Category: Hydrolase-hydrolase inhibitor complex]]~~	+
-	~~[[Category: Lysosome]]~~	+
-	~~[[Category: Molecular chaperone]]~~	+
-	~~[[Category: Pharmacological chaperone]]~~	+
-	~~[[Category: Protein binding~~]]	+

OCA at 08:18, 22 November 2017

2017-11-22T08:18:58Z

←Older revision		Revision as of 08:18, 22 November 2017
Line 3:		Line 3:
	<StructureSection load='4nxs' size='340' side='right' caption='[[4nxs]], [[Resolution\|resolution]] 2.55Å' scene=''>		<StructureSection load='4nxs' size='340' side='right' caption='[[4nxs]], [[Resolution\|resolution]] 2.55Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4nxs]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4NXS OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4NXS FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4nxs]] 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=4NXS OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4NXS FirstGlance]. <br>
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</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=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
		+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">GLA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Alpha-galactosidase Alpha-galactosidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.22 3.2.1.22] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Alpha-galactosidase Alpha-galactosidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.22 3.2.1.22] </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=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [http://pdbe.org/4nxs PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [http://www.ebi.ac.uk/pdbsum/4nxs PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4nxs 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=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [http://pdbe.org/4nxs PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [http://www.ebi.ac.uk/pdbsum/4nxs PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4nxs ProSAT]</span></td></tr>
Line 19:		Line 20:
	</div>		</div>
	<div class="pdbe-citations 4nxs" style="background-color:#fffaf0;"></div>		<div class="pdbe-citations 4nxs" style="background-color:#fffaf0;"></div>
-
-	==See Also==
-	*[[Galactosidase\|Galactosidase]]
	== References ==		== References ==
	<references/>		<references/>
Line 27:		Line 25:
	</StructureSection>		</StructureSection>
	[[Category: Alpha-galactosidase]]		[[Category: Alpha-galactosidase]]
		+	[[Category: Human]]
	[[Category: Drury, J E]]		[[Category: Drury, J E]]
	[[Category: Johnson, J L]]		[[Category: Johnson, J L]]
Line 36:		Line 35:
	[[Category: Fabry disease]]		[[Category: Fabry disease]]
	[[Category: Gla gene]]		[[Category: Gla gene]]
-	[[Category: Human]]
	[[Category: Hydrolase-hydrolase inhibitor complex]]		[[Category: Hydrolase-hydrolase inhibitor complex]]
	[[Category: Lysosome]]		[[Category: Lysosome]]

OCA at 18:53, 5 August 2016

2016-08-05T18:53:53Z

←Older revision		Revision as of 18:53, 5 August 2016
Line 1:		Line 1:
		+
	==Crystal structure of human alpha-galactosidase A in complex with 1-deoxygalactonojirimycin-pFPhT==		==Crystal structure of human alpha-galactosidase A in complex with 1-deoxygalactonojirimycin-pFPhT==
	<StructureSection load='4nxs' size='340' side='right' caption='[[4nxs]], [[Resolution\|resolution]] 2.55Å' scene=''>		<StructureSection load='4nxs' size='340' side='right' caption='[[4nxs]], [[Resolution\|resolution]] 2.55Å' scene=''>
Line 5:		Line 6:
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</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=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</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'>[http://en.wikipedia.org/wiki/Alpha-galactosidase Alpha-galactosidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.22 3.2.1.22] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Alpha-galactosidase Alpha-galactosidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.22 3.2.1.22] </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=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [http://www.ebi.ac.uk/pdbsum/4nxs 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=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [http://pdbe.org/4nxs PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [http://www.ebi.ac.uk/pdbsum/4nxs PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4nxs ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 17:		Line 18:
	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 4nxs" style="background-color:#fffaf0;"></div>
		+
		+	==See Also==
		+	*[[Galactosidase\|Galactosidase]]
	== References ==		== References ==
	<references/>		<references/>

OCA at 04:59, 25 December 2014

2014-12-25T04:59:25Z

←Older revision		Revision as of 04:59, 25 December 2014
Line 3:		Line 3:
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[4nxs]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4NXS OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4NXS FirstGlance]. <br>		<table><tr><td colspan='2'>[[4nxs]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4NXS OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4NXS FirstGlance]. <br>
-	</td></tr><tr><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</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=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
-	<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Alpha-galactosidase Alpha-galactosidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.22 3.2.1.22] </span></td></tr>	+	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Alpha-galactosidase Alpha-galactosidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.22 3.2.1.22] </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=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [http://www.ebi.ac.uk/pdbsum/4nxs 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=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [http://www.ebi.ac.uk/pdbsum/4nxs PDBsum]</span></td></tr>
-	<table>	+	</table>
	== Disease ==		== Disease ==
	[[http://www.uniprot.org/uniprot/AGAL_HUMAN AGAL_HUMAN]] Defects in GLA are the cause of Fabry disease (FD) [MIM:[http://omim.org/entry/301500 301500]]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.<ref>PMID:2152885</ref> <ref>PMID:1846223</ref> <ref>PMID:2171331</ref> <ref>PMID:2539398</ref> <ref>PMID:1315715</ref> <ref>PMID:7504405</ref> <ref>PMID:8395937</ref> <ref>PMID:8069316</ref> <ref>PMID:7531540</ref> <ref>PMID:7575533</ref> <ref>PMID:7759078</ref> <ref>PMID:7599642</ref> <ref>PMID:7596372</ref> <ref>PMID:8738659</ref> <ref>PMID:8875188</ref> <ref>PMID:8834244</ref> <ref>PMID:8931708</ref> <ref>PMID:8807334</ref> <ref>PMID:8863162</ref> <ref>PMID:9105656</ref> <ref>PMID:9100224</ref> <ref>PMID:9554750</ref> <ref>PMID:9452068</ref> <ref>PMID:9452090</ref> <ref>PMID:9452111</ref> <ref>PMID:10208848</ref> <ref>PMID:10090526</ref> <ref>PMID:10838196</ref> <ref>PMID:10666480</ref> <ref>PMID:11076046</ref> <ref>PMID:10916280</ref> <ref>PMID:11295840</ref> <ref>PMID:11668641</ref> <ref>PMID:11889412</ref> <ref>PMID:12694230</ref> <ref>PMID:12786754</ref> <ref>PMID:15162124</ref> <ref>PMID:15712228</ref> <ref>PMID:16533976</ref> <ref>PMID:19621417</ref>		[[http://www.uniprot.org/uniprot/AGAL_HUMAN AGAL_HUMAN]] Defects in GLA are the cause of Fabry disease (FD) [MIM:[http://omim.org/entry/301500 301500]]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.<ref>PMID:2152885</ref> <ref>PMID:1846223</ref> <ref>PMID:2171331</ref> <ref>PMID:2539398</ref> <ref>PMID:1315715</ref> <ref>PMID:7504405</ref> <ref>PMID:8395937</ref> <ref>PMID:8069316</ref> <ref>PMID:7531540</ref> <ref>PMID:7575533</ref> <ref>PMID:7759078</ref> <ref>PMID:7599642</ref> <ref>PMID:7596372</ref> <ref>PMID:8738659</ref> <ref>PMID:8875188</ref> <ref>PMID:8834244</ref> <ref>PMID:8931708</ref> <ref>PMID:8807334</ref> <ref>PMID:8863162</ref> <ref>PMID:9105656</ref> <ref>PMID:9100224</ref> <ref>PMID:9554750</ref> <ref>PMID:9452068</ref> <ref>PMID:9452090</ref> <ref>PMID:9452111</ref> <ref>PMID:10208848</ref> <ref>PMID:10090526</ref> <ref>PMID:10838196</ref> <ref>PMID:10666480</ref> <ref>PMID:11076046</ref> <ref>PMID:10916280</ref> <ref>PMID:11295840</ref> <ref>PMID:11668641</ref> <ref>PMID:11889412</ref> <ref>PMID:12694230</ref> <ref>PMID:12786754</ref> <ref>PMID:15162124</ref> <ref>PMID:15712228</ref> <ref>PMID:16533976</ref> <ref>PMID:19621417</ref>
-	== Function ==
-
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
Line 24:		Line 22:
	</StructureSection>		</StructureSection>
	[[Category: Alpha-galactosidase]]		[[Category: Alpha-galactosidase]]
-	[[Category: Drury, J E.]]	+	[[Category: Drury, J E]]
-	[[Category: Johnson, J L.]]	+	[[Category: Johnson, J L]]
-	[[Category: Lieberman, R L~~.]]~~	+	[[Category: Lieberman, R L]]
-	~~[[Category: Alpha-galactosidase~~]]	+
	[[Category: Arylthiourea]]		[[Category: Arylthiourea]]
	[[Category: Drug delivery system]]		[[Category: Drug delivery system]]

OCA at 08:44, 11 June 2014

2014-06-11T08:44:36Z

←Older revision		Revision as of 08:44, 11 June 2014
Line 1:		Line 1:
-	'''~~Unreleased~~ structure'''	+	==Crystal structure of human alpha-galactosidase A in complex with 1-deoxygalactonojirimycin-pFPhT==
		+	<StructureSection load='4nxs' size='340' side='right' caption='[[4nxs]], [[Resolution\|resolution]] 2.55Å' scene=''>
		+	== Structural highlights ==
		+	<table><tr><td colspan='2'>[[4nxs]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4NXS OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4NXS FirstGlance]. <br>
		+	</td></tr><tr><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=2OZ:(2R,3S,4R,5S)-N-(4-FLUOROPHENYL)-3,4,5-TRIHYDROXY-2-(HYDROXYMETHYL)PIPERIDINE-1-CARBOTHIOAMIDE'>2OZ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene><br>
		+	<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Alpha-galactosidase Alpha-galactosidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.22 3.2.1.22] </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=4nxs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4nxs OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4nxs RCSB], [http://www.ebi.ac.uk/pdbsum/4nxs PDBsum]</span></td></tr>
		+	<table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/AGAL_HUMAN AGAL_HUMAN]] Defects in GLA are the cause of Fabry disease (FD) [MIM:[http://omim.org/entry/301500 301500]]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.<ref>PMID:2152885</ref> <ref>PMID:1846223</ref> <ref>PMID:2171331</ref> <ref>PMID:2539398</ref> <ref>PMID:1315715</ref> <ref>PMID:7504405</ref> <ref>PMID:8395937</ref> <ref>PMID:8069316</ref> <ref>PMID:7531540</ref> <ref>PMID:7575533</ref> <ref>PMID:7759078</ref> <ref>PMID:7599642</ref> <ref>PMID:7596372</ref> <ref>PMID:8738659</ref> <ref>PMID:8875188</ref> <ref>PMID:8834244</ref> <ref>PMID:8931708</ref> <ref>PMID:8807334</ref> <ref>PMID:8863162</ref> <ref>PMID:9105656</ref> <ref>PMID:9100224</ref> <ref>PMID:9554750</ref> <ref>PMID:9452068</ref> <ref>PMID:9452090</ref> <ref>PMID:9452111</ref> <ref>PMID:10208848</ref> <ref>PMID:10090526</ref> <ref>PMID:10838196</ref> <ref>PMID:10666480</ref> <ref>PMID:11076046</ref> <ref>PMID:10916280</ref> <ref>PMID:11295840</ref> <ref>PMID:11668641</ref> <ref>PMID:11889412</ref> <ref>PMID:12694230</ref> <ref>PMID:12786754</ref> <ref>PMID:15162124</ref> <ref>PMID:15712228</ref> <ref>PMID:16533976</ref> <ref>PMID:19621417</ref>
		+	== Function ==

-	~~The entry 4nxs is ON HOLD until Paper~~ Publication	+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene often leading to missense alpha-galactosidase A (alpha-Gal A) variants that undergo premature endoplasmic reticulum-associated degradation due to folding defects. We have synthesized and characterized a new family of neutral amphiphilic pharmacological chaperones, namely 1-deoxygalactonojirimycin-arylthioureas (DGJ-ArTs), capable of stabilizing alpha-Gal A and restoring trafficking. Binding to the enzyme is reinforced by a strong hydrogen bond involving the aryl-N'H thiourea proton and the catalytic aspartic acid acid D231 of alpha-Gal A, as confirmed by a 2.55 A resolution cocrystal structure. Selected candidates enhanced alpha-Gal A activity and ameliorate globotriaosylceramide (Gb3) accumulation and autophagy impairments in FD cell cultures. Moreover, they acted synergistically with the proteostasis regulator 4-phenylbutyric acid, appearing to be promising leads as pharmacological chaperones for FD.

-	~~Authors~~: ~~Johnson, J.L~~., Drury, J.E., ~~Lieberman~~, R.L.	+	Molecular Basis of 1-Deoxygalactonojirimycin Arylthiourea Binding to Human alpha-Galactosidase A: Pharmacological Chaperoning Efficacy on Fabry Disease Mutants.,Yu Y, Mena-Barragan T, Higaki K, Johnson JL, Drury JE, Lieberman RL, Nakasone N, Ninomiya H, Tsukimura T, Sakuraba H, Suzuki Y, Nanba E, Mellet CO, Garcia Fernandez JM, Ohno K ACS Chem Biol. 2014 May 12. PMID:24783948<ref>PMID:24783948</ref>

-	~~Description: Crystal structure~~ of ~~human alpha~~-galactosidase ~~A in complex with 1~~-~~deoxygalactonojirimycin~~-~~pFPhT~~	+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
		+	</div>
		+	== References ==
		+	<references/>
		+	__TOC__
		+	</StructureSection>
		+	[[Category: Alpha-galactosidase]]
		+	[[Category: Drury, J E.]]
		+	[[Category: Johnson, J L.]]
		+	[[Category: Lieberman, R L.]]
		+	[[Category: Alpha-galactosidase]]
		+	[[Category: Arylthiourea]]
		+	[[Category: Drug delivery system]]
		+	[[Category: Enzyme activation]]
		+	[[Category: Enzyme stability]]
		+	[[Category: Fabry disease]]
		+	[[Category: Gla gene]]
		+	[[Category: Human]]
		+	[[Category: Hydrolase-hydrolase inhibitor complex]]
		+	[[Category: Lysosome]]
		+	[[Category: Molecular chaperone]]
		+	[[Category: Pharmacological chaperone]]
		+	[[Category: Protein binding]]

OCA at 09:20, 8 January 2014

2014-01-08T09:20:00Z

←Older revision		Revision as of 09:20, 8 January 2014
Line 1:		Line 1:
	'''Unreleased structure'''		'''Unreleased structure'''

-	The entry 4nxs is ON HOLD	+	The entry 4nxs is ON HOLD until Paper Publication

	Authors: Johnson, J.L., Drury, J.E., Lieberman, R.L.		Authors: Johnson, J.L., Drury, J.E., Lieberman, R.L.

	Description: Crystal structure of human alpha-galactosidase A in complex with 1-deoxygalactonojirimycin-pFPhT		Description: Crystal structure of human alpha-galactosidase A in complex with 1-deoxygalactonojirimycin-pFPhT

OCA: Protected "4nxs" [edit=sysop:move=sysop]

2013-12-18T09:26:18Z

Protected "4nxs" [edit=sysop:move=sysop]

←Older revision

Revision as of 09:26, 18 December 2013

OCA: New page: '''Unreleased structure''' The entry 4nxs is ON HOLD Authors: Johnson, J.L., Drury, J.E., Lieberman, R.L. Description: Crystal structure of human alpha-galactosidase A in complex with ...

2013-12-18T09:26:16Z

New page: '''Unreleased structure''' The entry 4nxs is ON HOLD Authors: Johnson, J.L., Drury, J.E., Lieberman, R.L. Description: Crystal structure of human alpha-galactosidase A in complex with ...

New page

'''Unreleased structure'''

The entry 4nxs is ON HOLD

Authors: Johnson, J.L., Drury, J.E., Lieberman, R.L.

Description: Crystal structure of human alpha-galactosidase A in complex with 1-deoxygalactonojirimycin-pFPhT