5hsh - Revision history

OCA at 10:52, 16 August 2023

OCA — Wed, 16 Aug 2023 10:52:48 GMT

←Older revision		Revision as of 10:52, 16 August 2023
Line 3:		Line 3:
	<StructureSection load='5hsh' size='340' side='right'caption='[[5hsh]], [[Resolution\|resolution]] 2.65Å' scene=''>		<StructureSection load='5hsh' size='340' side='right'caption='[[5hsh]], [[Resolution\|resolution]] 2.65Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[5hsh]] 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=5HSH OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=5HSH FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[5hsh]] 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=5HSH OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=5HSH FirstGlance]. <br>
-	</td></tr><tr id='~~ligand~~'><td class="sblockLbl"><b>[[~~Ligand~~\|~~Ligands~~:]]</b></td><td class="sblockDat"~~><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]] 2.65Å</td></tr>
-	~~<tr~~ id~~='related'><td class~~="~~sblockLbl~~">~~<b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[5epc\|5epc]]~~, [[~~5f9c~~\|~~5f9c~~]]</td></tr>	+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
-	<tr id='~~gene~~'><td class="sblockLbl"><b>[[~~Gene~~\|~~Gene~~:]]</b></td><td class="sblockDat"~~>PGM1 ([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=5hsh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5hsh OCA], [https://pdbe.org/5hsh PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=5hsh RCSB], [https://www.ebi.ac.uk/pdbsum/5hsh PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=5hsh 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/Phosphoglucomutase Phosphoglucomutase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=5.4.2.2 5.4.2.2]~~ </~~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=5hsh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5hsh OCA], [~~http~~://pdbe.org/5hsh PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=5hsh RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/5hsh PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=5hsh ProSAT]</span></td></tr>	+
	</table>		</table>
	== Disease ==		== Disease ==
-	[~~[http~~://www.uniprot.org/uniprot/PGM1_HUMAN PGM1_HUMAN]] PGM-CDG;Glycogen storage disease due to phosphoglucomutase deficiency. The disease is caused by mutations affecting the gene represented in this entry.	+	[https://www.uniprot.org/uniprot/PGM1_HUMAN PGM1_HUMAN] PGM-CDG;Glycogen storage disease due to phosphoglucomutase deficiency. The disease is caused by mutations affecting the gene represented in this entry.
	== Function ==		== Function ==
-	[~~[http~~://www.uniprot.org/uniprot/PGM1_HUMAN PGM1_HUMAN]] This enzyme participates in both the breakdown and synthesis of glucose.	+	[https://www.uniprot.org/uniprot/PGM1_HUMAN PGM1_HUMAN] This enzyme participates in both the breakdown and synthesis of glucose.
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
Line 27:		Line 25:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Human~~]]	+	[[Category: Homo sapiens]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	~~[[Category: Phosphoglucomutase]]~~	+	[[Category: Beamer LJ]]
-	[[Category: Beamer~~, L J~~]]	+	[[Category: Stiers KM]]
-	[[Category: Stiers~~, K M]]~~	+
-	~~[[Category: Enzyme]]~~	+
-	~~[[Category: Isomerase]]~~	+
-	~~[[Category: Missense mutant~~]]	+

OCA at 07:36, 27 November 2019

OCA — Wed, 27 Nov 2019 07:36:14 GMT

←Older revision		Revision as of 07:36, 27 November 2019
Line 1:		Line 1:

	==Crystal structure of the G291R mutant of human phosphoglucomutase 1==		==Crystal structure of the G291R mutant of human phosphoglucomutase 1==
-	<StructureSection load='5hsh' size='340' side='right' caption='[[5hsh]], [[Resolution\|resolution]] 2.65Å' scene=''>	+	<StructureSection load='5hsh' size='340' side='right'caption='[[5hsh]], [[Resolution\|resolution]] 2.65Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[5hsh]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5HSH OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5HSH FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[5hsh]] 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=5HSH OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5HSH FirstGlance]. <br>
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><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=SO4:SULFATE+ION'>SO4</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[5epc\|5epc]], [[5f9c\|5f9c]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[5epc\|5epc]], [[5f9c\|5f9c]]</td></tr>
		+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">PGM1 ([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/Phosphoglucomutase Phosphoglucomutase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=5.4.2.2 5.4.2.2] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Phosphoglucomutase Phosphoglucomutase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=5.4.2.2 5.4.2.2] </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=5hsh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5hsh OCA], [http://pdbe.org/5hsh PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5hsh RCSB], [http://www.ebi.ac.uk/pdbsum/5hsh PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5hsh 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=5hsh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5hsh OCA], [http://pdbe.org/5hsh PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5hsh RCSB], [http://www.ebi.ac.uk/pdbsum/5hsh PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5hsh ProSAT]</span></td></tr>
Line 26:		Line 27:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
		+	[[Category: Human]]
		+	[[Category: Large Structures]]
	[[Category: Phosphoglucomutase]]		[[Category: Phosphoglucomutase]]
	[[Category: Beamer, L J]]		[[Category: Beamer, L J]]

OCA at 10:51, 13 September 2017

OCA — Wed, 13 Sep 2017 10:51:04 GMT

←Older revision		Revision as of 10:51, 13 September 2017
Line 7:		Line 7:
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[5epc\|5epc]], [[5f9c\|5f9c]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[5epc\|5epc]], [[5f9c\|5f9c]]</td></tr>
	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Phosphoglucomutase Phosphoglucomutase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=5.4.2.2 5.4.2.2] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Phosphoglucomutase Phosphoglucomutase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=5.4.2.2 5.4.2.2] </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=5hsh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5hsh OCA], [http://pdbe.org/5hsh PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5hsh RCSB], [http://www.ebi.ac.uk/pdbsum/5hsh 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=5hsh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5hsh OCA], [http://pdbe.org/5hsh PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5hsh RCSB], [http://www.ebi.ac.uk/pdbsum/5hsh PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5hsh ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==

OCA at 12:06, 13 May 2016

OCA — Fri, 13 May 2016 12:06:50 GMT

←Older revision		Revision as of 12:06, 13 May 2016
Line 1:		Line 1:
-	'''Unreleased structure'''

-	The entry ~~5hsh~~ is ~~ON HOLD~~	+	==Crystal structure of the G291R mutant of human phosphoglucomutase 1==
		+	<StructureSection load='5hsh' size='340' side='right' caption='[[5hsh]], [[Resolution\|resolution]] 2.65Å' scene=''>
		+	== Structural highlights ==
		+	<table><tr><td colspan='2'>[[5hsh]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5HSH OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5HSH FirstGlance]. <br>
		+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><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">[[5epc\|5epc]], [[5f9c\|5f9c]]</td></tr>
		+	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Phosphoglucomutase Phosphoglucomutase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=5.4.2.2 5.4.2.2] </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=5hsh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5hsh OCA], [http://pdbe.org/5hsh PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5hsh RCSB], [http://www.ebi.ac.uk/pdbsum/5hsh PDBsum]</span></td></tr>
		+	</table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/PGM1_HUMAN PGM1_HUMAN]] PGM-CDG;Glycogen storage disease due to phosphoglucomutase deficiency. The disease is caused by mutations affecting the gene represented in this entry.
		+	== Function ==
		+	[[http://www.uniprot.org/uniprot/PGM1_HUMAN PGM1_HUMAN]] This enzyme participates in both the breakdown and synthesis of glucose.
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	Human phosphoglucomutase 1 (PGM1) plays a central role in cellular glucose homeostasis, mediating the switch between glycolysis and gluconeogenesis through the conversion of glucose 1-phosphate and glucose 6-phosphate. Recent clinical studies have identified mutations in this enzyme as the cause of PGM1 deficiency, an inborn error of metabolism classified as both a glycogen storage disease and a congenital disorder of glycosylation. Reported here are the first crystal structures of two disease-related missense variants of PGM1, along with the structure of the wild-type enzyme. Two independent glycine-to-arginine substitutions (G121R and G291R), both affecting key active site loops of PGM1, are found to induce regions of structural disorder, as evidenced by a nearly complete loss of electron density for as many as 23 aa. The disordered regions are not contiguous in sequence to the site of mutation, and even cross domain boundaries. Other structural rearrangements include changes in the conformations of loops and side chains, some of which occur nearly 20A away from the site of mutation. The induced structural disorder is correlated with increased sensitivity to proteolysis and lower-resolution diffraction, particularly for the G291R variant. Examination of the multi-domain effects of these GR mutations establishes a correlation between interdomain interfaces of the enzyme and missense variants of PGM1 associated with disease. These crystal structures provide the first insights into the structural basis of enzyme dysfunction in PGM1 deficiency and highlight a growing role for biophysical characterization of proteins in the field of precision medicine.

-	~~Authors:~~ Stiers, ~~K.M.~~, ~~Beamer~~, L.J.	+	Induced Structural Disorder as a Molecular Mechanism for Enzyme Dysfunction in Phosphoglucomutase 1 Deficiency.,Stiers KM, Kain BN, Graham AC, Beamer LJ J Mol Biol. 2016 Apr 24;428(8):1493-505. doi: 10.1016/j.jmb.2016.02.032. Epub, 2016 Mar 10. PMID:26972339<ref>PMID:26972339</ref>

-	~~Description: Crystal structure~~ of the ~~G291R mutant~~ of ~~human phosphoglucomutase 1~~	+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
-	[[Category: ~~Unreleased Structures~~]]	+	</div>
-	[[Category: Beamer, L.J]]	+	<div class="pdbe-citations 5hsh" style="background-color:#fffaf0;"></div>
-	[[Category: Stiers, K.M]]	+	== References ==
		+	<references/>
		+	__TOC__
		+	</StructureSection>
		+	[[Category: Phosphoglucomutase]]
		+	[[Category: Beamer, L J]]
		+	[[Category: Stiers, K M]]
		+	[[Category: Enzyme]]
		+	[[Category: Isomerase]]
		+	[[Category: Missense mutant]]

OCA at 12:58, 2 March 2016

OCA — Wed, 02 Mar 2016 12:58:28 GMT

←Older revision		Revision as of 12:58, 2 March 2016
Line 1:		Line 1:
	'''Unreleased structure'''		'''Unreleased structure'''

-	The entry 5hsh is ON HOLD ~~until Paper Publication~~	+	The entry 5hsh is ON HOLD

	Authors: Stiers, K.M., Beamer, L.J.		Authors: Stiers, K.M., Beamer, L.J.

OCA at 01:07, 21 February 2016

OCA — Sun, 21 Feb 2016 01:07:26 GMT

←Older revision		Revision as of 01:07, 21 February 2016
Line 1:		Line 1:
	'''Unreleased structure'''		'''Unreleased structure'''

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

	Authors: Stiers, K.M., Beamer, L.J.		Authors: Stiers, K.M., Beamer, L.J.

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

OCA — Wed, 03 Feb 2016 14:16:26 GMT

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

←Older revision

Revision as of 14:16, 3 February 2016

OCA: New page: '''Unreleased structure''' The entry 5hsh is ON HOLD Authors: Stiers, K.M., Beamer, L.J. Description: Crystal structure of the G291R mutant of human phosphoglucomutase 1 [[Category: Un...

OCA — Wed, 03 Feb 2016 14:16:24 GMT

New page: '''Unreleased structure''' The entry 5hsh is ON HOLD Authors: Stiers, K.M., Beamer, L.J. Description: Crystal structure of the G291R mutant of human phosphoglucomutase 1 [[Category: Un...

New page

'''Unreleased structure'''

The entry 5hsh is ON HOLD

Authors: Stiers, K.M., Beamer, L.J.

Description: Crystal structure of the G291R mutant of human phosphoglucomutase 1
[[Category: Unreleased Structures]]
[[Category: Beamer, L.J]]
[[Category: Stiers, K.M]]