4obs - Revision history

OCA at 17:09, 20 September 2023

2023-09-20T17:09:27Z

←Older revision		Revision as of 17:09, 20 September 2023
Line 4:		Line 4:
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[4obs]] is a 1 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=4OBS OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4OBS FirstGlance]. <br>		<table><tr><td colspan='2'>[[4obs]] is a 1 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=4OBS OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4OBS FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</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]] 2.26Å</td></tr>
		+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</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=4obs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4obs OCA], [https://pdbe.org/4obs PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4obs RCSB], [https://www.ebi.ac.uk/pdbsum/4obs PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4obs 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=4obs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4obs OCA], [https://pdbe.org/4obs PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4obs RCSB], [https://www.ebi.ac.uk/pdbsum/4obs PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4obs ProSAT]</span></td></tr>
	</table>		</table>

OCA at 07:15, 25 January 2023

2023-01-25T07:15:56Z

←Older revision		Revision as of 07:15, 25 January 2023
Line 3:		Line 3:
	<StructureSection load='4obs' size='340' side='right'caption='[[4obs]], [[Resolution\|resolution]] 2.26Å' scene=''>		<StructureSection load='4obs' size='340' side='right'caption='[[4obs]], [[Resolution\|resolution]] 2.26Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4obs]] is a 1 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=4OBS OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=4OBS FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4obs]] is a 1 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=4OBS OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4OBS FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</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=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
-	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4mj2\|4mj2]], [[4mj4\|4mj4]], [[4kgj\|4kgj]], [[4kh2\|4kh2]], [[4kgl\|4kgl]], [[4obr\|4obr]]</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=4obs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4obs OCA], [https://pdbe.org/4obs PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4obs RCSB], [https://www.ebi.ac.uk/pdbsum/4obs PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4obs ProSAT]</span></td></tr>
-	~~<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">IDUA ([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/L-iduronidase L-iduronidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.76 3.2.1.76] </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=4obs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4obs OCA], [~~http~~://pdbe.org/4obs PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=4obs RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/4obs PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=4obs ProSAT]</span></td></tr>	+
	</table>		</table>
	== Disease ==		== Disease ==
-	[~~[http~~://www.uniprot.org/uniprot/IDUA_HUMAN IDUA_HUMAN]] Hurler-Scheie syndrome;Hurler syndrome;Scheie syndrome. Defects in IDUA are the cause of mucopolysaccharidosis type 1H (MPS1H) [MIM:[~~http~~://omim.org/entry/607014 607014]]; also known as Hurler syndrome. MPS1H is a severe form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1H usually present, within the first year of life, a combination of hepatosplenomegaly, skeletal deformities, corneal clouding and severe mental retardation. Obstructive airways disease, respiratory infection and cardiac complications usually result in death before 10 years of age.<ref>PMID:8019563</ref> <ref>PMID:8401515</ref> <ref>PMID:8328452</ref> <ref>PMID:1301941</ref> <ref>PMID:7951228</ref> <ref>PMID:7550232</ref> <ref>PMID:7550242</ref> <ref>PMID:1946389</ref> <ref>PMID:10466419</ref> <ref>PMID:10735634</ref> <ref>PMID:12559846</ref> <ref>PMID:15300847</ref> <ref>PMID:21394825</ref> Defects in IDUA are the cause of mucopolysaccharidosis type 1H/S (MPS1H/S) [MIM:[~~http~~://omim.org/entry/607015 607015]]; also known as Hurler-Scheie syndrome. MPS1H/S is a form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. MPS1H/S represents an intermediate phenotype of the MPS1 clinical spectrum. It is characterized by relatively little neurological involvement, but most of the somatic symptoms described for severe MPS1 develop in the early to mid-teens, causing considerable loss of mobility. Defects in IDUA are the cause of mucopolysaccharidosis type 1S (MPS1S) [MIM:[~~http~~://omim.org/entry/607016 607016]]; also known as Scheie syndrome. MPS1S is a mild form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1S may have little or no neurological involvement, normal stature and life span, but present development of joints stiffness, mild hepatosplenomegaly, aortic valve disease and corneal clouding.	+	[https://www.uniprot.org/uniprot/IDUA_HUMAN IDUA_HUMAN] Hurler-Scheie syndrome;Hurler syndrome;Scheie syndrome. Defects in IDUA are the cause of mucopolysaccharidosis type 1H (MPS1H) [MIM:[https://omim.org/entry/607014 607014]; also known as Hurler syndrome. MPS1H is a severe form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1H usually present, within the first year of life, a combination of hepatosplenomegaly, skeletal deformities, corneal clouding and severe mental retardation. Obstructive airways disease, respiratory infection and cardiac complications usually result in death before 10 years of age.<ref>PMID:8019563</ref> <ref>PMID:8401515</ref> <ref>PMID:8328452</ref> <ref>PMID:1301941</ref> <ref>PMID:7951228</ref> <ref>PMID:7550232</ref> <ref>PMID:7550242</ref> <ref>PMID:1946389</ref> <ref>PMID:10466419</ref> <ref>PMID:10735634</ref> <ref>PMID:12559846</ref> <ref>PMID:15300847</ref> <ref>PMID:21394825</ref> Defects in IDUA are the cause of mucopolysaccharidosis type 1H/S (MPS1H/S) [MIM:[https://omim.org/entry/607015 607015]; also known as Hurler-Scheie syndrome. MPS1H/S is a form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. MPS1H/S represents an intermediate phenotype of the MPS1 clinical spectrum. It is characterized by relatively little neurological involvement, but most of the somatic symptoms described for severe MPS1 develop in the early to mid-teens, causing considerable loss of mobility. Defects in IDUA are the cause of mucopolysaccharidosis type 1S (MPS1S) [MIM:[https://omim.org/entry/607016 607016]; also known as Scheie syndrome. MPS1S is a mild form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1S may have little or no neurological involvement, normal stature and life span, but present development of joints stiffness, mild hepatosplenomegaly, aortic valve disease and corneal clouding.
		+	== Function ==
		+	[https://www.uniprot.org/uniprot/IDUA_HUMAN IDUA_HUMAN]
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Human]]~~	+	[[Category: Homo sapiens]]
-	~~[[Category: L-iduronidase~~]]	+
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	[[Category: Bie, H]]	+	[[Category: Bie H]]
-	[[Category: He, X]]	+	[[Category: He X]]
-	[[Category: James~~, M N.G~~]]	+	[[Category: James MNG]]
-	[[Category: Kermode~~, A R~~]]	+	[[Category: Kermode AR]]
-	[[Category: Yin, J]]	+	[[Category: Yin J]]
-	~~[[Category: Beta sandwich]]~~	+
-	~~[[Category: Fibronectin iii domain]]~~	+
-	~~[[Category: Glycosaminoglycan]]~~	+
-	~~[[Category: Glycoside hydrolase family 39]]~~	+
-	~~[[Category: Hydrolase]]~~	+
-	~~[[Category: Tim barrel~~]]	+

OCA at 08:33, 10 April 2019

2019-04-10T08:33:40Z

←Older revision		Revision as of 08:33, 10 April 2019
Line 1:		Line 1:

	==Crystal structure of human alpha-L-iduronidase in the P212121 form==		==Crystal structure of human alpha-L-iduronidase in the P212121 form==
-	<StructureSection load='4obs' size='340' side='right' caption='[[4obs]], [[Resolution\|resolution]] 2.26Å' scene=''>	+	<StructureSection load='4obs' size='340' side='right'caption='[[4obs]], [[Resolution\|resolution]] 2.26Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4obs]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4OBS OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4OBS FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4obs]] is a 1 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=4OBS OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4OBS FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=~~NAG~~:~~N-ACETYL~~-D-~~GLUCOSAMINE~~'>~~NAG~~</scene>, <scene name='pdbligand=~~SO4~~:~~SULFATE+ION~~'>~~SO4~~</scene>, <scene name='pdbligand=~~MAN~~:~~ALPHA-D-MANNOSE~~'>~~MAN~~</scene></td></tr>	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4mj2\|4mj2]], [[4mj4\|4mj4]], [[4kgj\|4kgj]], [[4kh2\|4kh2]], [[4kgl\|4kgl]], [[4obr\|4obr]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4mj2\|4mj2]], [[4mj4\|4mj4]], [[4kgj\|4kgj]], [[4kh2\|4kh2]], [[4kgl\|4kgl]], [[4obr\|4obr]]</td></tr>
		+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">IDUA ([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/L-iduronidase L-iduronidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.76 3.2.1.76] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/L-iduronidase L-iduronidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.76 3.2.1.76] </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=4obs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4obs OCA], [http://pdbe.org/4obs PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4obs RCSB], [http://www.ebi.ac.uk/pdbsum/4obs PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4obs 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=4obs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4obs OCA], [http://pdbe.org/4obs PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4obs RCSB], [http://www.ebi.ac.uk/pdbsum/4obs PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4obs ProSAT]</span></td></tr>
Line 15:		Line 16:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
		+	[[Category: Human]]
	[[Category: L-iduronidase]]		[[Category: L-iduronidase]]
		+	[[Category: Large Structures]]
	[[Category: Bie, H]]		[[Category: Bie, H]]
	[[Category: He, X]]		[[Category: He, X]]

OCA at 18:02, 11 August 2016

2016-08-11T18:02:32Z

←Older revision		Revision as of 18:02, 11 August 2016
Line 1:		Line 1:
		+
	==Crystal structure of human alpha-L-iduronidase in the P212121 form==		==Crystal structure of human alpha-L-iduronidase in the P212121 form==
	<StructureSection load='4obs' size='340' side='right' caption='[[4obs]], [[Resolution\|resolution]] 2.26Å' scene=''>		<StructureSection load='4obs' size='340' side='right' caption='[[4obs]], [[Resolution\|resolution]] 2.26Å' scene=''>
Line 6:		Line 7:
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4mj2\|4mj2]], [[4mj4\|4mj4]], [[4kgj\|4kgj]], [[4kh2\|4kh2]], [[4kgl\|4kgl]], [[4obr\|4obr]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4mj2\|4mj2]], [[4mj4\|4mj4]], [[4kgj\|4kgj]], [[4kh2\|4kh2]], [[4kgl\|4kgl]], [[4obr\|4obr]]</td></tr>
	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/L-iduronidase L-iduronidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.76 3.2.1.76] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/L-iduronidase L-iduronidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.76 3.2.1.76] </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=4obs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4obs OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4obs RCSB], [http://www.ebi.ac.uk/pdbsum/4obs 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=4obs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4obs OCA], [http://pdbe.org/4obs PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4obs RCSB], [http://www.ebi.ac.uk/pdbsum/4obs PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4obs ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==

OCA at 13:41, 14 January 2015

2015-01-14T13:41:32Z

←Older revision		Revision as of 13:41, 14 January 2015
Line 1:		Line 1:
-	'''~~Unreleased structure~~'''	+	==Crystal structure of human alpha-L-iduronidase in the P212121 form==
-		+	<StructureSection load='4obs' size='340' side='right' caption='[[4obs]], [[Resolution\|resolution]] 2.26Å' scene=''>
-	~~The entry~~ 4obs is ~~ON HOLD until Paper Publication~~	+	== Structural highlights ==
-		+	<table><tr><td colspan='2'>[[4obs]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4OBS OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4OBS FirstGlance]. <br>
-	~~Authors~~: ~~Bie~~, H., ~~Yin~~, J., He, X., ~~Kermode~~, A.R., ~~James~~, M.N.G.	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</scene></td></tr>
-		+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4mj2\|4mj2]], [[4mj4\|4mj4]], [[4kgj\|4kgj]], [[4kh2\|4kh2]], [[4kgl\|4kgl]], [[4obr\|4obr]]</td></tr>
-	~~Description~~: ~~Crystal structure~~ of ~~human alpha~~-L-~~iduronidase~~ in the ~~P212121~~ form	+	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/L-iduronidase L-iduronidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.76 3.2.1.76] </span></td></tr>
-	[[Category: ~~Unreleased Structures~~]]	+	<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=4obs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4obs OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4obs RCSB], [http://www.ebi.ac.uk/pdbsum/4obs PDBsum]</span></td></tr>
		+	</table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/IDUA_HUMAN IDUA_HUMAN]] Hurler-Scheie syndrome;Hurler syndrome;Scheie syndrome. Defects in IDUA are the cause of mucopolysaccharidosis type 1H (MPS1H) [MIM:[http://omim.org/entry/607014 607014]]; also known as Hurler syndrome. MPS1H is a severe form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1H usually present, within the first year of life, a combination of hepatosplenomegaly, skeletal deformities, corneal clouding and severe mental retardation. Obstructive airways disease, respiratory infection and cardiac complications usually result in death before 10 years of age.<ref>PMID:8019563</ref> <ref>PMID:8401515</ref> <ref>PMID:8328452</ref> <ref>PMID:1301941</ref> <ref>PMID:7951228</ref> <ref>PMID:7550232</ref> <ref>PMID:7550242</ref> <ref>PMID:1946389</ref> <ref>PMID:10466419</ref> <ref>PMID:10735634</ref> <ref>PMID:12559846</ref> <ref>PMID:15300847</ref> <ref>PMID:21394825</ref> Defects in IDUA are the cause of mucopolysaccharidosis type 1H/S (MPS1H/S) [MIM:[http://omim.org/entry/607015 607015]]; also known as Hurler-Scheie syndrome. MPS1H/S is a form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. MPS1H/S represents an intermediate phenotype of the MPS1 clinical spectrum. It is characterized by relatively little neurological involvement, but most of the somatic symptoms described for severe MPS1 develop in the early to mid-teens, causing considerable loss of mobility. Defects in IDUA are the cause of mucopolysaccharidosis type 1S (MPS1S) [MIM:[http://omim.org/entry/607016 607016]]; also known as Scheie syndrome. MPS1S is a mild form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1S may have little or no neurological involvement, normal stature and life span, but present development of joints stiffness, mild hepatosplenomegaly, aortic valve disease and corneal clouding.
		+	== References ==
		+	<references/>
		+	__TOC__
		+	</StructureSection>
		+	[[Category: L-iduronidase]]
	[[Category: Bie, H]]		[[Category: Bie, H]]
	[[Category: He, X]]		[[Category: He, X]]
-	[[Category: ~~Kermode~~, A.R]]	+	[[Category: James, M N.G]]
-	[[Category: ~~James~~, ~~M.N.G~~]]	+	[[Category: Kermode, A R]]
	[[Category: Yin, J]]		[[Category: Yin, J]]
		+	[[Category: Beta sandwich]]
		+	[[Category: Fibronectin iii domain]]
		+	[[Category: Glycosaminoglycan]]
		+	[[Category: Glycoside hydrolase family 39]]
		+	[[Category: Hydrolase]]
		+	[[Category: Tim barrel]]

OCA at 14:21, 24 December 2014

2014-12-24T14:21:37Z

←Older revision		Revision as of 14:21, 24 December 2014
Line 6:		Line 6:

	Description: Crystal structure of human alpha-L-iduronidase in the P212121 form		Description: Crystal structure of human alpha-L-iduronidase in the P212121 form
		+	[[Category: Unreleased Structures]]
		+	[[Category: Bie, H]]
		+	[[Category: He, X]]
		+	[[Category: Kermode, A.R]]
		+	[[Category: James, M.N.G]]
		+	[[Category: Yin, J]]

OCA at 10:26, 5 February 2014

2014-02-05T10:26:44Z

←Older revision		Revision as of 10:26, 5 February 2014
Line 1:		Line 1:
	'''Unreleased structure'''		'''Unreleased structure'''

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

	Authors: Bie, H., Yin, J., He, X., Kermode, A.R., James, M.N.G.		Authors: Bie, H., Yin, J., He, X., Kermode, A.R., James, M.N.G.

	Description: Crystal structure of human alpha-L-iduronidase in the P212121 form		Description: Crystal structure of human alpha-L-iduronidase in the P212121 form

OCA at 06:46, 22 January 2014

2014-01-22T06:46:57Z

←Older revision		Revision as of 06:46, 22 January 2014
Line 5:		Line 5:
	Authors: Bie, H., Yin, J., He, X., Kermode, A.R., James, M.N.G.		Authors: Bie, H., Yin, J., He, X., Kermode, A.R., James, M.N.G.

-	Description: ~~crystal~~ structure of human alpha-L-iduronidase in the P212121 form	+	Description: Crystal structure of human alpha-L-iduronidase in the P212121 form

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

2014-01-15T07:45:35Z

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

←Older revision

Revision as of 07:45, 15 January 2014

OCA: New page: '''Unreleased structure''' The entry 4obs is ON HOLD Authors: Bie, H., Yin, J., He, X., Kermode, A.R., James, M.N.G. Description: crystal structure of human alpha-L-iduronidase in the ...

2014-01-15T07:45:35Z

New page: '''Unreleased structure''' The entry 4obs is ON HOLD Authors: Bie, H., Yin, J., He, X., Kermode, A.R., James, M.N.G. Description: crystal structure of human alpha-L-iduronidase in the ...

New page

'''Unreleased structure'''

The entry 4obs is ON HOLD

Authors: Bie, H., Yin, J., He, X., Kermode, A.R., James, M.N.G.

Description: crystal structure of human alpha-L-iduronidase in the P212121 form