4m0u - Revision history

OCA at 14:39, 8 November 2023

OCA — Wed, 08 Nov 2023 14:39:41 GMT

←Older revision		Revision as of 14:39, 8 November 2023
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	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[4m0u]] 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=4M0U OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4M0U FirstGlance]. <br>		<table><tr><td colspan='2'>[[4m0u]] 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=4M0U OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4M0U FirstGlance]. <br>
-	</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>	+	</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models\|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution\|Resolution]] 2.74Å</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='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=4m0u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4m0u OCA], [https://pdbe.org/4m0u PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4m0u RCSB], [https://www.ebi.ac.uk/pdbsum/4m0u PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4m0u 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=4m0u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4m0u OCA], [https://pdbe.org/4m0u PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4m0u RCSB], [https://www.ebi.ac.uk/pdbsum/4m0u PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4m0u ProSAT]</span></td></tr>
	</table>		</table>

OCA at 10:45, 21 December 2022

OCA — Wed, 21 Dec 2022 10:45:51 GMT

←Older revision		Revision as of 10:45, 21 December 2022
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	<StructureSection load='4m0u' size='340' side='right'caption='[[4m0u]], [[Resolution\|resolution]] 2.74Å' scene=''>		<StructureSection load='4m0u' size='340' side='right'caption='[[4m0u]], [[Resolution\|resolution]] 2.74Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4m0u]] 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=4M0U OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=4M0U FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4m0u]] 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=4M0U OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4M0U 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" id="ligandDat"><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">[[4lyg\|4lyg]], [[4lzp\|4lzp]], [[4lzo\|4lzo]], [[4m0p\|4m0p]]</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=4m0u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4m0u OCA], [https://pdbe.org/4m0u PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4m0u RCSB], [https://www.ebi.ac.uk/pdbsum/4m0u PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4m0u ProSAT]</span></td></tr>
-	~~<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">PRPS1 ([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/Ribose-phosphate_diphosphokinase Ribose-phosphate diphosphokinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.6.1 2.7.6.1] </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=4m0u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4m0u OCA], [~~http~~://pdbe.org/4m0u PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=4m0u RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/4m0u PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=4m0u ProSAT]</span></td></tr>	+
	</table>		</table>
	== Disease ==		== Disease ==
-	[~~[http~~://www.uniprot.org/uniprot/PRPS1_HUMAN PRPS1_HUMAN]] Defects in PRPS1 are the cause of phosphoribosylpyrophosphate synthetase superactivity (PRPS1 superactivity) [MIM:[~~http~~://omim.org/entry/300661 300661]]; also known as PRPS-related gout. It is a familial disorder characterized by excessive purine production, gout and uric acid urolithiasis. Defects in PRPS1 are the cause of Charcot-Marie-Tooth disease X-linked recessive type 5 (CMTX5) [MIM:[~~http~~://omim.org/entry/311070 311070]]; also known as optic atrophy-polyneuropathy-deafness or Rosenberg-Chutorian syndrome. CMTX5 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy.<ref>PMID:17701900</ref> Defects in PRPS1 are the cause of ARTS syndrome (ARTS) [MIM:[~~http~~://omim.org/entry/301835 301835]]; also known as fatal ataxia X-linked with deafness and loss of vision. ARTS is a disorder characterized by mental retardation, early-onset hypotonia, ataxia, delayed motor development, hearing impairment, and optic atrophy. Susceptibility to infections, especially of the upper respiratory tract, can result in early death.<ref>PMID:17701896</ref> Defects in PRPS1 are the cause of deafness X-linked type 1 (DFNX1) [MIM:[~~http~~://omim.org/entry/304500 304500]]; also known as congenital sensorineural deafness X-linked 2 (DFN2). It is a form of deafness characterized by progressive, severe-to-profound sensorineural hearing loss in males. Females manifest mild to moderate hearing loss.<ref>PMID:20021999</ref>	+	[https://www.uniprot.org/uniprot/PRPS1_HUMAN PRPS1_HUMAN] Defects in PRPS1 are the cause of phosphoribosylpyrophosphate synthetase superactivity (PRPS1 superactivity) [MIM:[https://omim.org/entry/300661 300661]; also known as PRPS-related gout. It is a familial disorder characterized by excessive purine production, gout and uric acid urolithiasis. Defects in PRPS1 are the cause of Charcot-Marie-Tooth disease X-linked recessive type 5 (CMTX5) [MIM:[https://omim.org/entry/311070 311070]; also known as optic atrophy-polyneuropathy-deafness or Rosenberg-Chutorian syndrome. CMTX5 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy.<ref>PMID:17701900</ref> Defects in PRPS1 are the cause of ARTS syndrome (ARTS) [MIM:[https://omim.org/entry/301835 301835]; also known as fatal ataxia X-linked with deafness and loss of vision. ARTS is a disorder characterized by mental retardation, early-onset hypotonia, ataxia, delayed motor development, hearing impairment, and optic atrophy. Susceptibility to infections, especially of the upper respiratory tract, can result in early death.<ref>PMID:17701896</ref> Defects in PRPS1 are the cause of deafness X-linked type 1 (DFNX1) [MIM:[https://omim.org/entry/304500 304500]; also known as congenital sensorineural deafness X-linked 2 (DFN2). It is a form of deafness characterized by progressive, severe-to-profound sensorineural hearing loss in males. Females manifest mild to moderate hearing loss.<ref>PMID:20021999</ref>
	== Function ==		== Function ==
-	[~~[http~~://www.uniprot.org/uniprot/PRPS1_HUMAN PRPS1_HUMAN]] Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.	+	[https://www.uniprot.org/uniprot/PRPS1_HUMAN PRPS1_HUMAN] Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
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	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Human~~]]	+	[[Category: Homo sapiens]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	~~[[Category: Ribose-phosphate diphosphokinase]]~~	+	[[Category: Chen P]]
-	[[Category: Chen, P]]	+	[[Category: Li X]]
-	[[Category: Li, X]]	+	[[Category: Teng M]]
-	[[Category: Teng, M]]	+
-	~~[[Category: Atp r5p]]~~	+
-	~~[[Category: Prpp synthesis enzyme]]~~	+
-	~~[[Category: Prs1]]~~	+
-	~~[[Category: Transferase~~]]	+

OCA at 08:35, 3 April 2019

OCA — Wed, 03 Apr 2019 08:35:17 GMT

←Older revision		Revision as of 08:35, 3 April 2019
Line 1:		Line 1:

	==crystal structure of human PRS1 Q133P mutant==		==crystal structure of human PRS1 Q133P mutant==
-	<StructureSection load='4m0u' size='340' side='right' caption='[[4m0u]], [[Resolution\|resolution]] 2.74Å' scene=''>	+	<StructureSection load='4m0u' size='340' side='right'caption='[[4m0u]], [[Resolution\|resolution]] 2.74Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4m0u]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4M0U OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4M0U FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4m0u]] 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=4M0U OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4M0U 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">[[4lyg\|4lyg]], [[4lzp\|4lzp]], [[4lzo\|4lzo]], [[4m0p\|4m0p]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4lyg\|4lyg]], [[4lzp\|4lzp]], [[4lzo\|4lzo]], [[4m0p\|4m0p]]</td></tr>
		+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">PRPS1 ([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/Ribose-phosphate_diphosphokinase Ribose-phosphate diphosphokinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.6.1 2.7.6.1] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Ribose-phosphate_diphosphokinase Ribose-phosphate diphosphokinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.6.1 2.7.6.1] </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=4m0u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4m0u OCA], [http://pdbe.org/4m0u PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4m0u RCSB], [http://www.ebi.ac.uk/pdbsum/4m0u PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4m0u 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=4m0u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4m0u OCA], [http://pdbe.org/4m0u PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4m0u RCSB], [http://www.ebi.ac.uk/pdbsum/4m0u PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4m0u ProSAT]</span></td></tr>
Line 26:		Line 27:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
		+	[[Category: Human]]
		+	[[Category: Large Structures]]
	[[Category: Ribose-phosphate diphosphokinase]]		[[Category: Ribose-phosphate diphosphokinase]]
	[[Category: Chen, P]]		[[Category: Chen, P]]

OCA at 16:51, 2 January 2017

OCA — Mon, 02 Jan 2017 16:51:36 GMT

←Older revision		Revision as of 16:51, 2 January 2017
Line 1:		Line 1:
		+
	==crystal structure of human PRS1 Q133P mutant==		==crystal structure of human PRS1 Q133P mutant==
	<StructureSection load='4m0u' size='340' side='right' caption='[[4m0u]], [[Resolution\|resolution]] 2.74Å' scene=''>		<StructureSection load='4m0u' size='340' side='right' caption='[[4m0u]], [[Resolution\|resolution]] 2.74Å' scene=''>
Line 6:		Line 7:
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4lyg\|4lyg]], [[4lzp\|4lzp]], [[4lzo\|4lzo]], [[4m0p\|4m0p]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4lyg\|4lyg]], [[4lzp\|4lzp]], [[4lzo\|4lzo]], [[4m0p\|4m0p]]</td></tr>
	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Ribose-phosphate_diphosphokinase Ribose-phosphate diphosphokinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.6.1 2.7.6.1] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Ribose-phosphate_diphosphokinase Ribose-phosphate diphosphokinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.6.1 2.7.6.1] </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=4m0u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4m0u OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4m0u RCSB], [http://www.ebi.ac.uk/pdbsum/4m0u 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=4m0u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4m0u OCA], [http://pdbe.org/4m0u PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4m0u RCSB], [http://www.ebi.ac.uk/pdbsum/4m0u PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4m0u ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 20:		Line 21:
	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 4m0u" style="background-color:#fffaf0;"></div>
	== References ==		== References ==
	<references/>		<references/>

OCA at 07:20, 26 March 2015

OCA — Thu, 26 Mar 2015 07:20:42 GMT

←Older revision		Revision as of 07:20, 26 March 2015
Line 12:		Line 12:
	== Function ==		== Function ==
	[[http://www.uniprot.org/uniprot/PRPS1_HUMAN PRPS1_HUMAN]] Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.		[[http://www.uniprot.org/uniprot/PRPS1_HUMAN PRPS1_HUMAN]] Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	Human PRS1, which is indispensable for the biosynthesis of nucleotides, deoxynucleotides and their derivatives, is associated directly with multiple human diseases because of single base mutation. However, a molecular understanding of the effect of these mutations is hampered by the lack of understanding of its catalytic mechanism. Here, we reconstruct the 3D EM structure of the PRS1 apo state. Together with the native stain EM structures of AMPNPP, AMPNPP and R5P, ADP and the apo states with distinct conformations, we suggest the hexamer is the enzymatically active form. Based on crystal structures, sequence analysis, mutagenesis, enzyme kinetics assays, and MD simulations, we reveal the conserved substrates binding motifs and make further analysis of all pathogenic mutants.
		+
		+	Crystal and EM Structures of Human Phosphoribosyl Pyrophosphate Synthase I (PRS1) Provide Novel Insights into the Disease-Associated Mutations.,Chen P, Liu Z, Wang X, Peng J, Sun Q, Li J, Wang M, Niu L, Zhang Z, Cai G, Teng M, Li X PLoS One. 2015 Mar 17;10(3):e0120304. doi: 10.1371/journal.pone.0120304., eCollection 2015. PMID:25781187<ref>PMID:25781187</ref>
		+
		+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
		+	</div>
	== References ==		== References ==
	<references/>		<references/>

OCA at 15:54, 7 February 2015

OCA — Sat, 07 Feb 2015 15:54:43 GMT

←Older revision		Revision as of 15:54, 7 February 2015
Line 1:		Line 1:
-	'''~~Unreleased structure~~'''	+	==crystal structure of human PRS1 Q133P mutant==
-		+	<StructureSection load='4m0u' size='340' side='right' caption='[[4m0u]], [[Resolution\|resolution]] 2.74Å' scene=''>
-	~~The entry~~ 4m0u is ~~ON HOLD until Feb 02 2016~~	+	== Structural highlights ==
-		+	<table><tr><td colspan='2'>[[4m0u]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4M0U OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4M0U FirstGlance]. <br>
-	~~Authors~~: ~~Chen~~, P., ~~Teng~~, M., Li, X.	+	</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">[[4lyg\|4lyg]], [[4lzp\|4lzp]], [[4lzo\|4lzo]], [[4m0p\|4m0p]]</td></tr>
-	~~Description~~: ~~crystal structure~~ of ~~human PRS1 Q133P mutant~~	+	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Ribose-phosphate_diphosphokinase Ribose-phosphate diphosphokinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.6.1 2.7.6.1] </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=4m0u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4m0u OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4m0u RCSB], [http://www.ebi.ac.uk/pdbsum/4m0u PDBsum]</span></td></tr>
		+	</table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/PRPS1_HUMAN PRPS1_HUMAN]] Defects in PRPS1 are the cause of phosphoribosylpyrophosphate synthetase superactivity (PRPS1 superactivity) [MIM:[http://omim.org/entry/300661 300661]]; also known as PRPS-related gout. It is a familial disorder characterized by excessive purine production, gout and uric acid urolithiasis. Defects in PRPS1 are the cause of Charcot-Marie-Tooth disease X-linked recessive type 5 (CMTX5) [MIM:[http://omim.org/entry/311070 311070]]; also known as optic atrophy-polyneuropathy-deafness or Rosenberg-Chutorian syndrome. CMTX5 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy.<ref>PMID:17701900</ref> Defects in PRPS1 are the cause of ARTS syndrome (ARTS) [MIM:[http://omim.org/entry/301835 301835]]; also known as fatal ataxia X-linked with deafness and loss of vision. ARTS is a disorder characterized by mental retardation, early-onset hypotonia, ataxia, delayed motor development, hearing impairment, and optic atrophy. Susceptibility to infections, especially of the upper respiratory tract, can result in early death.<ref>PMID:17701896</ref> Defects in PRPS1 are the cause of deafness X-linked type 1 (DFNX1) [MIM:[http://omim.org/entry/304500 304500]]; also known as congenital sensorineural deafness X-linked 2 (DFN2). It is a form of deafness characterized by progressive, severe-to-profound sensorineural hearing loss in males. Females manifest mild to moderate hearing loss.<ref>PMID:20021999</ref>
		+	== Function ==
		+	[[http://www.uniprot.org/uniprot/PRPS1_HUMAN PRPS1_HUMAN]] Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.
		+	== References ==
		+	<references/>
		+	__TOC__
		+	</StructureSection>
		+	[[Category: Ribose-phosphate diphosphokinase]]
	[[Category: Chen, P]]		[[Category: Chen, P]]
-	[[Category: Teng, M]]
	[[Category: Li, X]]		[[Category: Li, X]]
		+	[[Category: Teng, M]]
		+	[[Category: Atp r5p]]
		+	[[Category: Prpp synthesis enzyme]]
		+	[[Category: Prs1]]
		+	[[Category: Transferase]]

OCA at 14:03, 24 December 2014

OCA — Wed, 24 Dec 2014 14:03:41 GMT

←Older revision		Revision as of 14:03, 24 December 2014
Line 6:		Line 6:

	Description: crystal structure of human PRS1 Q133P mutant		Description: crystal structure of human PRS1 Q133P mutant
		+	[[Category: Unreleased Structures]]
		+	[[Category: Chen, P]]
		+	[[Category: Teng, M]]
		+	[[Category: Li, X]]

OCA at 00:42, 7 August 2014

OCA — Thu, 07 Aug 2014 00:42:59 GMT

←Older revision		Revision as of 00:42, 7 August 2014
Line 1:		Line 1:
	'''Unreleased structure'''		'''Unreleased structure'''

-	The entry 4m0u is ON HOLD until ~~Aug~~ 02 ~~2015~~	+	The entry 4m0u is ON HOLD until Feb 02 2016

	Authors: Chen, P., Teng, M., Li, X.		Authors: Chen, P., Teng, M., Li, X.

	Description: crystal structure of human PRS1 Q133P mutant		Description: crystal structure of human PRS1 Q133P mutant

OCA at 04:46, 12 September 2013

OCA — Thu, 12 Sep 2013 04:46:21 GMT

←Older revision		Revision as of 04:46, 12 September 2013
Line 1:		Line 1:
	'''Unreleased structure'''		'''Unreleased structure'''

-	The entry 4m0u is ON HOLD	+	The entry 4m0u is ON HOLD until Aug 02 2015

	Authors: Chen, P., Teng, M., Li, X.		Authors: Chen, P., Teng, M., Li, X.

	Description: crystal structure of human PRS1 Q133P mutant		Description: crystal structure of human PRS1 Q133P mutant

OCA at 06:50, 28 August 2013

OCA — Wed, 28 Aug 2013 06:50:22 GMT

←Older revision		Revision as of 06:50, 28 August 2013
Line 3:		Line 3:
	The entry 4m0u is ON HOLD		The entry 4m0u is ON HOLD

-	Authors: ~~Peng~~ Chen, ~~Maikun~~ Teng, ~~Xu LI~~	+	Authors: Chen, P., Teng, M., Li, X.

	Description: crystal structure of human PRS1 Q133P mutant		Description: crystal structure of human PRS1 Q133P mutant