4mri - Revision history

OCA at 16:41, 20 September 2023

2023-09-20T16:41:12Z

←Older revision		Revision as of 16:41, 20 September 2023
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	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[4mri]] 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=4MRI OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4MRI FirstGlance]. <br>		<table><tr><td colspan='2'>[[4mri]] 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=4MRI OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4MRI FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=AS9:N-[HYDROXY(METHYL)PHOSPHORYL]-L-ASPARTIC+ACID'>AS9</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</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]] 2.8Å</td></tr>
		+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=AS9:N-[HYDROXY(METHYL)PHOSPHORYL]-L-ASPARTIC+ACID'>AS9</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [https://pdbe.org/4mri PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [https://www.ebi.ac.uk/pdbsum/4mri PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4mri 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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [https://pdbe.org/4mri PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [https://www.ebi.ac.uk/pdbsum/4mri PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4mri ProSAT]</span></td></tr>
	</table>		</table>

OCA at 10:03, 28 December 2022

2022-12-28T10:03:38Z

←Older revision		Revision as of 10:03, 28 December 2022
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	==Human brain aspartoacylase mutant F295S complex with intermediate analog (N-phosphonomethyl-L-aspartate)==		==Human brain aspartoacylase mutant F295S complex with intermediate analog (N-phosphonomethyl-L-aspartate)==
-	<StructureSection load='4mri' size='340' side='right' caption='[[4mri]], [[Resolution\|resolution]] 2.80Å' scene=''>	+	<StructureSection load='4mri' size='340' side='right'caption='[[4mri]], [[Resolution\|resolution]] 2.80Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4mri]] 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=4MRI OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=4MRI FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4mri]] 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=4MRI OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4MRI FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=AS9:N-[HYDROXY(METHYL)PHOSPHORYL]-L-ASPARTIC+ACID'>AS9</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene~~></td></tr>~~	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=AS9:N-[HYDROXY(METHYL)PHOSPHORYL]-L-ASPARTIC+ACID'>AS9</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
-	~~<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2o4h\|2o4h]], [[2o53\|2o53]]</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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [https://pdbe.org/4mri PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [https://www.ebi.ac.uk/pdbsum/4mri PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4mri ProSAT]</span></td></tr>
-	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">ACY2, ASP, ASPA ([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/Aspartoacylase Aspartoacylase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.5.1.15 3.5.1.15] </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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [~~http~~://pdbe.org/4mri PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/4mri PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=4mri ProSAT]</span></td></tr>	+
	</table>		</table>
	== Disease ==		== Disease ==
-	[~~[http~~://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN]] Defects in ASPA are the cause of Canavan disease (CAND) [MIM:[~~http~~://omim.org/entry/271900 271900]]; also known as spongy degeneration of the brain. CAND is a rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. The clinical features are onset in early infancy, atonia of neck muscles, hypotonia, hyperextension of legs and flexion of arms, blindness, severe mental defect, megalocephaly, and death by 18 months on the average.<ref>PMID:8252036</ref> <ref>PMID:12706335</ref> <ref>PMID:8023850</ref> <ref>PMID:7668285</ref> <ref>PMID:7599639</ref> <ref>PMID:8659549</ref> <ref>PMID:9452117</ref> <ref>PMID:10564886</ref> <ref>PMID:10407784</ref> <ref>PMID:10909858</ref> <ref>PMID:12638939</ref> <ref>PMID:12205125</ref>	+	[https://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN] Defects in ASPA are the cause of Canavan disease (CAND) [MIM:[https://omim.org/entry/271900 271900]; also known as spongy degeneration of the brain. CAND is a rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. The clinical features are onset in early infancy, atonia of neck muscles, hypotonia, hyperextension of legs and flexion of arms, blindness, severe mental defect, megalocephaly, and death by 18 months on the average.<ref>PMID:8252036</ref> <ref>PMID:12706335</ref> <ref>PMID:8023850</ref> <ref>PMID:7668285</ref> <ref>PMID:7599639</ref> <ref>PMID:8659549</ref> <ref>PMID:9452117</ref> <ref>PMID:10564886</ref> <ref>PMID:10407784</ref> <ref>PMID:10909858</ref> <ref>PMID:12638939</ref> <ref>PMID:12205125</ref>
	== Function ==		== Function ==
-	[~~[http~~://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN]] Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids.	+	[https://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN] Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids.
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
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	</div>		</div>
	<div class="pdbe-citations 4mri" style="background-color:#fffaf0;"></div>		<div class="pdbe-citations 4mri" style="background-color:#fffaf0;"></div>
		+
		+	==See Also==
		+	*[[Aminoacylase 3D structures\|Aminoacylase 3D structures]]
		+	*[[Aspartoacylase 3D structures\|Aspartoacylase 3D structures]]
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Aspartoacylase~~]]	+	[[Category: Homo sapiens]]
-	[[Category: ~~Human~~]]	+	[[Category: Large Structures]]
-	[[Category: Pavlovsky~~, A G~~]]	+	[[Category: Pavlovsky AG]]
-	[[Category: Viola~~, R E~~]]	+	[[Category: Viola RE]]
-	[[Category: Wijayasinghe~~, Y S]]~~	+	[[Category: Wijayasinghe YS]]
-	~~[[Category: Aminoacylase-2]]~~	+
-	~~[[Category: Aspartoacylase family]]~~	+
-	~~[[Category: Canavan disease]]~~	+
-	~~[[Category: Hydrolase]]~~	+
-	~~[[Category: N-acetyl-l-aspartate]]~~	+
-	~~[[Category: Zinc-dependent hydrolase~~]]	+

OCA at 09:55, 15 November 2017

2017-11-15T09:55:55Z

←Older revision		Revision as of 09:55, 15 November 2017
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	<StructureSection load='4mri' size='340' side='right' caption='[[4mri]], [[Resolution\|resolution]] 2.80Å' scene=''>		<StructureSection load='4mri' size='340' side='right' caption='[[4mri]], [[Resolution\|resolution]] 2.80Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4mri]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4MRI OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4MRI FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4mri]] 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=4MRI OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4MRI FirstGlance]. <br>
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=AS9:N-[HYDROXY(METHYL)PHOSPHORYL]-L-ASPARTIC+ACID'>AS9</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>		</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=AS9:N-[HYDROXY(METHYL)PHOSPHORYL]-L-ASPARTIC+ACID'>AS9</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2o4h\|2o4h]], [[2o53\|2o53]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2o4h\|2o4h]], [[2o53\|2o53]]</td></tr>
		+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">ACY2, ASP, ASPA ([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/Aspartoacylase Aspartoacylase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.5.1.15 3.5.1.15] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Aspartoacylase Aspartoacylase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.5.1.15 3.5.1.15] </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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [http://pdbe.org/4mri PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [http://www.ebi.ac.uk/pdbsum/4mri PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4mri 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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [http://pdbe.org/4mri PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [http://www.ebi.ac.uk/pdbsum/4mri PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4mri ProSAT]</span></td></tr>
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	</div>		</div>
	<div class="pdbe-citations 4mri" style="background-color:#fffaf0;"></div>		<div class="pdbe-citations 4mri" style="background-color:#fffaf0;"></div>
-
-	==See Also==
-	*[[Aspartoacylase\|Aspartoacylase]]
	== References ==		== References ==
	<references/>		<references/>
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	</StructureSection>		</StructureSection>
	[[Category: Aspartoacylase]]		[[Category: Aspartoacylase]]
		+	[[Category: Human]]
	[[Category: Pavlovsky, A G]]		[[Category: Pavlovsky, A G]]
	[[Category: Viola, R E]]		[[Category: Viola, R E]]

OCA at 19:04, 11 August 2016

2016-08-11T19:04:58Z

←Older revision		Revision as of 19:04, 11 August 2016
Line 1:		Line 1:
		+
	==Human brain aspartoacylase mutant F295S complex with intermediate analog (N-phosphonomethyl-L-aspartate)==		==Human brain aspartoacylase mutant F295S complex with intermediate analog (N-phosphonomethyl-L-aspartate)==
	<StructureSection load='4mri' size='340' side='right' caption='[[4mri]], [[Resolution\|resolution]] 2.80Å' scene=''>		<StructureSection load='4mri' size='340' side='right' caption='[[4mri]], [[Resolution\|resolution]] 2.80Å' scene=''>
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	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2o4h\|2o4h]], [[2o53\|2o53]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2o4h\|2o4h]], [[2o53\|2o53]]</td></tr>
	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Aspartoacylase Aspartoacylase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.5.1.15 3.5.1.15] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Aspartoacylase Aspartoacylase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.5.1.15 3.5.1.15] </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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [http://www.ebi.ac.uk/pdbsum/4mri 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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [http://pdbe.org/4mri PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [http://www.ebi.ac.uk/pdbsum/4mri PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4mri ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
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	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 4mri" style="background-color:#fffaf0;"></div>
		+
		+	==See Also==
		+	*[[Aspartoacylase\|Aspartoacylase]]
	== References ==		== References ==
	<references/>		<references/>

OCA at 14:38, 5 January 2015

2015-01-05T14:38:24Z

←Older revision		Revision as of 14:38, 5 January 2015
Line 3:		Line 3:
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[4mri]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4MRI OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4MRI FirstGlance]. <br>		<table><tr><td colspan='2'>[[4mri]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4MRI OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4MRI FirstGlance]. <br>
-	</td></tr><tr><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=AS9:N-[HYDROXY(METHYL)PHOSPHORYL]-L-ASPARTIC+ACID'>AS9</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene><br>	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=AS9:N-[HYDROXY(METHYL)PHOSPHORYL]-L-ASPARTIC+ACID'>AS9</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
-	<tr><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2o4h\|2o4h]], [[2o53\|2o53]]</td></tr>	+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2o4h\|2o4h]], [[2o53\|2o53]]</td></tr>
-	<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Aspartoacylase Aspartoacylase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.5.1.15 3.5.1.15] </span></td></tr>	+	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Aspartoacylase Aspartoacylase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.5.1.15 3.5.1.15] </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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [http://www.ebi.ac.uk/pdbsum/4mri 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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [http://www.ebi.ac.uk/pdbsum/4mri PDBsum]</span></td></tr>
-	<table>	+	</table>
	== Disease ==		== Disease ==
	[[http://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN]] Defects in ASPA are the cause of Canavan disease (CAND) [MIM:[http://omim.org/entry/271900 271900]]; also known as spongy degeneration of the brain. CAND is a rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. The clinical features are onset in early infancy, atonia of neck muscles, hypotonia, hyperextension of legs and flexion of arms, blindness, severe mental defect, megalocephaly, and death by 18 months on the average.<ref>PMID:8252036</ref> <ref>PMID:12706335</ref> <ref>PMID:8023850</ref> <ref>PMID:7668285</ref> <ref>PMID:7599639</ref> <ref>PMID:8659549</ref> <ref>PMID:9452117</ref> <ref>PMID:10564886</ref> <ref>PMID:10407784</ref> <ref>PMID:10909858</ref> <ref>PMID:12638939</ref> <ref>PMID:12205125</ref>		[[http://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN]] Defects in ASPA are the cause of Canavan disease (CAND) [MIM:[http://omim.org/entry/271900 271900]]; also known as spongy degeneration of the brain. CAND is a rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. The clinical features are onset in early infancy, atonia of neck muscles, hypotonia, hyperextension of legs and flexion of arms, blindness, severe mental defect, megalocephaly, and death by 18 months on the average.<ref>PMID:8252036</ref> <ref>PMID:12706335</ref> <ref>PMID:8023850</ref> <ref>PMID:7668285</ref> <ref>PMID:7599639</ref> <ref>PMID:8659549</ref> <ref>PMID:9452117</ref> <ref>PMID:10564886</ref> <ref>PMID:10407784</ref> <ref>PMID:10909858</ref> <ref>PMID:12638939</ref> <ref>PMID:12205125</ref>
Line 25:		Line 25:
	</StructureSection>		</StructureSection>
	[[Category: Aspartoacylase]]		[[Category: Aspartoacylase]]
-	[[Category: Pavlovsky, A G.]]	+	[[Category: Pavlovsky, A G]]
-	[[Category: Viola, R E.]]	+	[[Category: Viola, R E]]
-	[[Category: Wijayasinghe, Y S.]]	+	[[Category: Wijayasinghe, Y S]]
	[[Category: Aminoacylase-2]]		[[Category: Aminoacylase-2]]
	[[Category: Aspartoacylase family]]		[[Category: Aspartoacylase family]]

OCA at 07:32, 30 July 2014

2014-07-30T07:32:07Z

←Older revision		Revision as of 07:32, 30 July 2014
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-	'''~~Unreleased~~ structure'''	+	==Human brain aspartoacylase mutant F295S complex with intermediate analog (N-phosphonomethyl-L-aspartate)==
		+	<StructureSection load='4mri' size='340' side='right' caption='[[4mri]], [[Resolution\|resolution]] 2.80Å' scene=''>
		+	== Structural highlights ==
		+	<table><tr><td colspan='2'>[[4mri]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4MRI OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4MRI FirstGlance]. <br>
		+	</td></tr><tr><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=AS9:N-[HYDROXY(METHYL)PHOSPHORYL]-L-ASPARTIC+ACID'>AS9</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene><br>
		+	<tr><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[2o4h\|2o4h]], [[2o53\|2o53]]</td></tr>
		+	<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Aspartoacylase Aspartoacylase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.5.1.15 3.5.1.15] </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=4mri FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4mri OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4mri RCSB], [http://www.ebi.ac.uk/pdbsum/4mri PDBsum]</span></td></tr>
		+	<table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN]] Defects in ASPA are the cause of Canavan disease (CAND) [MIM:[http://omim.org/entry/271900 271900]]; also known as spongy degeneration of the brain. CAND is a rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. The clinical features are onset in early infancy, atonia of neck muscles, hypotonia, hyperextension of legs and flexion of arms, blindness, severe mental defect, megalocephaly, and death by 18 months on the average.<ref>PMID:8252036</ref> <ref>PMID:12706335</ref> <ref>PMID:8023850</ref> <ref>PMID:7668285</ref> <ref>PMID:7599639</ref> <ref>PMID:8659549</ref> <ref>PMID:9452117</ref> <ref>PMID:10564886</ref> <ref>PMID:10407784</ref> <ref>PMID:10909858</ref> <ref>PMID:12638939</ref> <ref>PMID:12205125</ref>
		+	== Function ==
		+	[[http://www.uniprot.org/uniprot/ACY2_HUMAN ACY2_HUMAN]] Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids.
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	Canavan disease (CD) is a fatal, childhood neurological disorder caused by mutations in the ASPA gene, leading to catalytic deficiencies in the aspartoacylase (ASPA) enzyme and impaired N-acetyl-l-aspartic acid metabolism in the brain. To study the possible structural defects triggered by these mutations, four ASPA missense mutations associated with different disease severities have been structurally characterized. These mutant enzymes each have overall structures similar to that of the native ASPA enzyme, but with varying degrees of alterations that offer explanations for the respective loss of catalytic activity. The K213E mutant, a nonconservative mutant associated with a mild disease phenotype, has minimal structural differences compared to the native enzyme. In contrast, the loss of van der Waals contacts in the F295S mutant and the loss of hydrophobic and hydrogen bonding interactions in the Y231C mutant lead to a local collapse of the hydrophobic core structure in the carboxyl-terminal domain, contributing to a decrease in protein stability. The structure of the E285A mutant, the most common clinical mutant, reveals that the loss of hydrogen bonding interactions with the carboxylate side chain of Glu285 disturbs the active site architecture, leading to altered substrate binding and lower catalytic activity. Our improved understanding of the nature of these structural defects provides a basis for the development of treatment therapies for CD.

-	~~The entry 4mri is ON HOLD until Paper Publication~~	+	Aspartoacylase Catalytic Deficiency as the Cause of Canavan Disease: A Structural Perspective.,Wijayasinghe YS, Pavlovsky AG, Viola RE Biochemistry. 2014 Jul 16. PMID:25003821<ref>PMID:25003821</ref>

-	~~Authors: Wijayasinghe~~, Y.S., Pavlovsky, A.G., Viola, R.E.	+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
-		+	</div>
-	~~Description~~: ~~Human brain aspartoacylase mutant F295S complex with intermediate analog (~~N-~~phosphonomethyl~~-L-aspartate)	+	== References ==
		+	<references/>
		+	__TOC__
		+	</StructureSection>
		+	[[Category: Aspartoacylase]]
		+	[[Category: Pavlovsky, A G.]]
		+	[[Category: Viola, R E.]]
		+	[[Category: Wijayasinghe, Y S.]]
		+	[[Category: Aminoacylase-2]]
		+	[[Category: Aspartoacylase family]]
		+	[[Category: Canavan disease]]
		+	[[Category: Hydrolase]]
		+	[[Category: N-acetyl-l-aspartate]]
		+	[[Category: Zinc-dependent hydrolase]]

OCA at 09:40, 27 November 2013

2013-11-27T09:40:11Z

←Older revision		Revision as of 09:40, 27 November 2013
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	'''Unreleased structure'''		'''Unreleased structure'''

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

	Authors: Wijayasinghe, Y.S., Pavlovsky, A.G., Viola, R.E.		Authors: Wijayasinghe, Y.S., Pavlovsky, A.G., Viola, R.E.

	Description: Human brain aspartoacylase mutant F295S complex with intermediate analog (N-phosphonomethyl-L-aspartate)		Description: Human brain aspartoacylase mutant F295S complex with intermediate analog (N-phosphonomethyl-L-aspartate)

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

2013-09-29T07:42:32Z

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

←Older revision

Revision as of 07:42, 29 September 2013

OCA: New page: '''Unreleased structure''' The entry 4mri is ON HOLD Authors: Wijayasinghe, Y.S., Pavlovsky, A.G., Viola, R.E. Description: Human brain aspartoacylase mutant F295S complex with interme...

2013-09-29T07:42:31Z

New page: '''Unreleased structure''' The entry 4mri is ON HOLD Authors: Wijayasinghe, Y.S., Pavlovsky, A.G., Viola, R.E. Description: Human brain aspartoacylase mutant F295S complex with interme...

New page

'''Unreleased structure'''

The entry 4mri is ON HOLD

Authors: Wijayasinghe, Y.S., Pavlovsky, A.G., Viola, R.E.

Description: Human brain aspartoacylase mutant F295S complex with intermediate analog (N-phosphonomethyl-L-aspartate)