4cj6 - Revision history

OCA at 12:11, 20 December 2023

OCA — Wed, 20 Dec 2023 12:11:08 GMT

←Older revision		Revision as of 12:11, 20 December 2023
Line 4:		Line 4:
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[4cj6]] 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=4CJ6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4CJ6 FirstGlance]. <br>		<table><tr><td colspan='2'>[[4cj6]] 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=4CJ6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4CJ6 FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=RET:RETINAL'>RET</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]] 1.896Å</td></tr>
		+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=RET:RETINAL'>RET</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=4cj6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cj6 OCA], [https://pdbe.org/4cj6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4cj6 RCSB], [https://www.ebi.ac.uk/pdbsum/4cj6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4cj6 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=4cj6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cj6 OCA], [https://pdbe.org/4cj6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4cj6 RCSB], [https://www.ebi.ac.uk/pdbsum/4cj6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4cj6 ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
-	[[https://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Defects in RLBP1 are a cause of retinitis pigmentosa autosomal recessive (ARRP) [MIM:[https://omim.org/entry/268000 268000]]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.<ref>PMID:9326942</ref> Defects in RLBP1 are the cause of Bothnia retinal dystrophy (BRD) [MIM:[https://omim.org/entry/607475 607475]]; also known as Vasterbotten dystrophy. Affected individuals show night blindness from early childhood with features consistent with retinitis punctata albescens and macular degeneration.<ref>PMID:10102298</ref> Defects in RLBP1 are the cause of rod-cone dystrophy Newfoundland (NFRCD) [MIM:[https://omim.org/entry/607476 607476]]. NFRCD is a retinal dystrophy reminiscent of retinitis punctata albescens but with a substantially lower age at onset and more-rapid and distinctive progression. Rod-cone dystrophies results from initial loss of rod photoreceptors, later followed by cone photoreceptors loss.<ref>PMID:11868161</ref> Defects in RLBP1 are a cause of retinitis punctata albescens (RPA) [MIM:[https://omim.org/entry/136880 136880]]. A rare form of stationary night blindness characterized by a delay in the regeneration of cone and rod photopigments.	+	[https://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN] Defects in RLBP1 are a cause of retinitis pigmentosa autosomal recessive (ARRP) [MIM:[https://omim.org/entry/268000 268000]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.<ref>PMID:9326942</ref> Defects in RLBP1 are the cause of Bothnia retinal dystrophy (BRD) [MIM:[https://omim.org/entry/607475 607475]; also known as Vasterbotten dystrophy. Affected individuals show night blindness from early childhood with features consistent with retinitis punctata albescens and macular degeneration.<ref>PMID:10102298</ref> Defects in RLBP1 are the cause of rod-cone dystrophy Newfoundland (NFRCD) [MIM:[https://omim.org/entry/607476 607476]. NFRCD is a retinal dystrophy reminiscent of retinitis punctata albescens but with a substantially lower age at onset and more-rapid and distinctive progression. Rod-cone dystrophies results from initial loss of rod photoreceptors, later followed by cone photoreceptors loss.<ref>PMID:11868161</ref> Defects in RLBP1 are a cause of retinitis punctata albescens (RPA) [MIM:[https://omim.org/entry/136880 136880]. A rare form of stationary night blindness characterized by a delay in the regeneration of cone and rod photopigments.
	== Function ==		== Function ==
-	[[https://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Soluble retinoid carrier essential the proper function of both rod and cone photoreceptors. Participates in the regeneration of active 11-cis-retinol and 11-cis-retinaldehyde, from the inactive 11-trans products of the rhodopsin photocycle and in the de novo synthesis of these retinoids from 11-trans metabolic precursors. The cycling of retinoids between photoreceptor and adjacent pigment epithelium cells is known as the 'visual cycle'.<ref>PMID:19846785</ref>	+	[https://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN] Soluble retinoid carrier essential the proper function of both rod and cone photoreceptors. Participates in the regeneration of active 11-cis-retinol and 11-cis-retinaldehyde, from the inactive 11-trans products of the rhodopsin photocycle and in the de novo synthesis of these retinoids from 11-trans metabolic precursors. The cycling of retinoids between photoreceptor and adjacent pigment epithelium cells is known as the 'visual cycle'.<ref>PMID:19846785</ref>
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==

OCA at 07:17, 14 September 2022

OCA — Wed, 14 Sep 2022 07:17:59 GMT

←Older revision		Revision as of 07:17, 14 September 2022
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	<StructureSection load='4cj6' size='340' side='right'caption='[[4cj6]], [[Resolution\|resolution]] 1.90Å' scene=''>		<StructureSection load='4cj6' size='340' side='right'caption='[[4cj6]], [[Resolution\|resolution]] 1.90Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[4cj6]] 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=4CJ6 OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=4CJ6 FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[4cj6]] 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=4CJ6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4CJ6 FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=RET:RETINAL'>RET</scene>~~</td></tr>~~	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=RET:RETINAL'>RET</scene></td></tr>
-	~~<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4ciz\|4ciz]]~~</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=4cj6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cj6 OCA], [https://pdbe.org/4cj6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4cj6 RCSB], [https://www.ebi.ac.uk/pdbsum/4cj6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4cj6 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=4cj6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cj6 OCA], [~~http~~://pdbe.org/4cj6 PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=4cj6 RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/4cj6 PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=4cj6 ProSAT]</span></td></tr>	+
	</table>		</table>
	== Disease ==		== Disease ==
-	[[~~http~~://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Defects in RLBP1 are a cause of retinitis pigmentosa autosomal recessive (ARRP) [MIM:[~~http~~://omim.org/entry/268000 268000]]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.<ref>PMID:9326942</ref> Defects in RLBP1 are the cause of Bothnia retinal dystrophy (BRD) [MIM:[~~http~~://omim.org/entry/607475 607475]]; also known as Vasterbotten dystrophy. Affected individuals show night blindness from early childhood with features consistent with retinitis punctata albescens and macular degeneration.<ref>PMID:10102298</ref> Defects in RLBP1 are the cause of rod-cone dystrophy Newfoundland (NFRCD) [MIM:[~~http~~://omim.org/entry/607476 607476]]. NFRCD is a retinal dystrophy reminiscent of retinitis punctata albescens but with a substantially lower age at onset and more-rapid and distinctive progression. Rod-cone dystrophies results from initial loss of rod photoreceptors, later followed by cone photoreceptors loss.<ref>PMID:11868161</ref> Defects in RLBP1 are a cause of retinitis punctata albescens (RPA) [MIM:[~~http~~://omim.org/entry/136880 136880]]. A rare form of stationary night blindness characterized by a delay in the regeneration of cone and rod photopigments.	+	[[https://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Defects in RLBP1 are a cause of retinitis pigmentosa autosomal recessive (ARRP) [MIM:[https://omim.org/entry/268000 268000]]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.<ref>PMID:9326942</ref> Defects in RLBP1 are the cause of Bothnia retinal dystrophy (BRD) [MIM:[https://omim.org/entry/607475 607475]]; also known as Vasterbotten dystrophy. Affected individuals show night blindness from early childhood with features consistent with retinitis punctata albescens and macular degeneration.<ref>PMID:10102298</ref> Defects in RLBP1 are the cause of rod-cone dystrophy Newfoundland (NFRCD) [MIM:[https://omim.org/entry/607476 607476]]. NFRCD is a retinal dystrophy reminiscent of retinitis punctata albescens but with a substantially lower age at onset and more-rapid and distinctive progression. Rod-cone dystrophies results from initial loss of rod photoreceptors, later followed by cone photoreceptors loss.<ref>PMID:11868161</ref> Defects in RLBP1 are a cause of retinitis punctata albescens (RPA) [MIM:[https://omim.org/entry/136880 136880]]. A rare form of stationary night blindness characterized by a delay in the regeneration of cone and rod photopigments.
	== Function ==		== Function ==
-	[[~~http~~://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Soluble retinoid carrier essential the proper function of both rod and cone photoreceptors. Participates in the regeneration of active 11-cis-retinol and 11-cis-retinaldehyde, from the inactive 11-trans products of the rhodopsin photocycle and in the de novo synthesis of these retinoids from 11-trans metabolic precursors. The cycling of retinoids between photoreceptor and adjacent pigment epithelium cells is known as the 'visual cycle'.<ref>PMID:19846785</ref>	+	[[https://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Soluble retinoid carrier essential the proper function of both rod and cone photoreceptors. Participates in the regeneration of active 11-cis-retinol and 11-cis-retinaldehyde, from the inactive 11-trans products of the rhodopsin photocycle and in the de novo synthesis of these retinoids from 11-trans metabolic precursors. The cycling of retinoids between photoreceptor and adjacent pigment epithelium cells is known as the 'visual cycle'.<ref>PMID:19846785</ref>
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
Line 25:		Line 24:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Human~~]]	+	[[Category: Homo sapiens]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	[[Category: Bolze~~, C S~~]]	+	[[Category: Bolze CS]]
-	[[Category: Cascella, M]]	+	[[Category: Cascella M]]
-	[[Category: Dworkowski, F]]	+	[[Category: Dworkowski F]]
-	[[Category: Fuchs~~, M R~~]]	+	[[Category: Fuchs MR]]
-	[[Category: Furrer, J]]	+	[[Category: Furrer J]]
-	[[Category: Golczak, M]]	+	[[Category: Golczak M]]
-	[[Category: Helbling~~, R E~~]]	+	[[Category: Helbling RE]]
-	[[Category: Owen~~, R L~~]]	+	[[Category: Owen RL]]
-	[[Category: Palczewski, K]]	+	[[Category: Palczewski K]]
-	[[Category: Pearson~~, A R~~]]	+	[[Category: Pearson AR]]
-	[[Category: Pompidor, G]]	+	[[Category: Pompidor G]]
-	[[Category: Stocker, A]]	+	[[Category: Stocker A]]
-	~~[[Category: 9-cis-retinal]]~~	+
-	~~[[Category: Isomerase]]~~	+
-	~~[[Category: Visual cycle~~]]	+

OCA at 09:53, 20 March 2019

OCA — Wed, 20 Mar 2019 09:53:05 GMT

←Older revision		Revision as of 09:53, 20 March 2019
Line 1:		Line 1:

	==Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal==		==Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal==
-	<StructureSection load='4cj6' size='340' side='right' caption='[[4cj6]], [[Resolution\|resolution]] 1.90Å' scene=''>	+	<StructureSection load='4cj6' size='340' side='right'caption='[[4cj6]], [[Resolution\|resolution]] 1.90Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[4cj6]] 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=4CJ6 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4CJ6 FirstGlance]. <br>		<table><tr><td colspan='2'>[[4cj6]] 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=4CJ6 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4CJ6 FirstGlance]. <br>
Line 26:		Line 26:
	</StructureSection>		</StructureSection>
	[[Category: Human]]		[[Category: Human]]
		+	[[Category: Large Structures]]
	[[Category: Bolze, C S]]		[[Category: Bolze, C S]]
	[[Category: Cascella, M]]		[[Category: Cascella, M]]

OCA at 20:22, 11 August 2016

OCA — Thu, 11 Aug 2016 20:22:52 GMT

←Older revision		Revision as of 20:22, 11 August 2016
Line 1:		Line 1:
		+
	==Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal==		==Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal==
	<StructureSection load='4cj6' size='340' side='right' caption='[[4cj6]], [[Resolution\|resolution]] 1.90Å' scene=''>		<StructureSection load='4cj6' size='340' side='right' caption='[[4cj6]], [[Resolution\|resolution]] 1.90Å' scene=''>
Line 5:		Line 6:
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=RET:RETINAL'>RET</scene></td></tr>		</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=RET:RETINAL'>RET</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4ciz\|4ciz]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4ciz\|4ciz]]</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=4cj6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cj6 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4cj6 RCSB], [http://www.ebi.ac.uk/pdbsum/4cj6 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=4cj6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cj6 OCA], [http://pdbe.org/4cj6 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4cj6 RCSB], [http://www.ebi.ac.uk/pdbsum/4cj6 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4cj6 ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 19:		Line 20:
	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 4cj6" style="background-color:#fffaf0;"></div>
	== References ==		== References ==
	<references/>		<references/>

OCA at 13:22, 4 January 2015

OCA — Sun, 04 Jan 2015 13:22:30 GMT

←Older revision		Revision as of 13:22, 4 January 2015
Line 1:		Line 1:
-	~~{{STRUCTURE_4cj6\| PDB=4cj6 \| SCENE= }}~~	+	==Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal==
-	===Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal===	+	<StructureSection load='4cj6' size='340' side='right' caption='[[4cj6]], [[Resolution\|resolution]] 1.90Å' scene=''>
-	~~{{ABSTRACT_PUBMED_24328211}}~~	+	== Structural highlights ==
-		+	<table><tr><td colspan='2'>[[4cj6]] 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=4CJ6 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4CJ6 FirstGlance]. <br>
-	==Disease==	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=RET:RETINAL'>RET</scene></td></tr>
		+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[4ciz\|4ciz]]</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=4cj6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cj6 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4cj6 RCSB], [http://www.ebi.ac.uk/pdbsum/4cj6 PDBsum]</span></td></tr>
		+	</table>
		+	== Disease ==
	[[http://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Defects in RLBP1 are a cause of retinitis pigmentosa autosomal recessive (ARRP) [MIM:[http://omim.org/entry/268000 268000]]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.<ref>PMID:9326942</ref> Defects in RLBP1 are the cause of Bothnia retinal dystrophy (BRD) [MIM:[http://omim.org/entry/607475 607475]]; also known as Vasterbotten dystrophy. Affected individuals show night blindness from early childhood with features consistent with retinitis punctata albescens and macular degeneration.<ref>PMID:10102298</ref> Defects in RLBP1 are the cause of rod-cone dystrophy Newfoundland (NFRCD) [MIM:[http://omim.org/entry/607476 607476]]. NFRCD is a retinal dystrophy reminiscent of retinitis punctata albescens but with a substantially lower age at onset and more-rapid and distinctive progression. Rod-cone dystrophies results from initial loss of rod photoreceptors, later followed by cone photoreceptors loss.<ref>PMID:11868161</ref> Defects in RLBP1 are a cause of retinitis punctata albescens (RPA) [MIM:[http://omim.org/entry/136880 136880]]. A rare form of stationary night blindness characterized by a delay in the regeneration of cone and rod photopigments.		[[http://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Defects in RLBP1 are a cause of retinitis pigmentosa autosomal recessive (ARRP) [MIM:[http://omim.org/entry/268000 268000]]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.<ref>PMID:9326942</ref> Defects in RLBP1 are the cause of Bothnia retinal dystrophy (BRD) [MIM:[http://omim.org/entry/607475 607475]]; also known as Vasterbotten dystrophy. Affected individuals show night blindness from early childhood with features consistent with retinitis punctata albescens and macular degeneration.<ref>PMID:10102298</ref> Defects in RLBP1 are the cause of rod-cone dystrophy Newfoundland (NFRCD) [MIM:[http://omim.org/entry/607476 607476]]. NFRCD is a retinal dystrophy reminiscent of retinitis punctata albescens but with a substantially lower age at onset and more-rapid and distinctive progression. Rod-cone dystrophies results from initial loss of rod photoreceptors, later followed by cone photoreceptors loss.<ref>PMID:11868161</ref> Defects in RLBP1 are a cause of retinitis punctata albescens (RPA) [MIM:[http://omim.org/entry/136880 136880]]. A rare form of stationary night blindness characterized by a delay in the regeneration of cone and rod photopigments.
-		+	== Function ==
-	==Function==	+
	[[http://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Soluble retinoid carrier essential the proper function of both rod and cone photoreceptors. Participates in the regeneration of active 11-cis-retinol and 11-cis-retinaldehyde, from the inactive 11-trans products of the rhodopsin photocycle and in the de novo synthesis of these retinoids from 11-trans metabolic precursors. The cycling of retinoids between photoreceptor and adjacent pigment epithelium cells is known as the 'visual cycle'.<ref>PMID:19846785</ref>		[[http://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Soluble retinoid carrier essential the proper function of both rod and cone photoreceptors. Participates in the regeneration of active 11-cis-retinol and 11-cis-retinaldehyde, from the inactive 11-trans products of the rhodopsin photocycle and in the de novo synthesis of these retinoids from 11-trans metabolic precursors. The cycling of retinoids between photoreceptor and adjacent pigment epithelium cells is known as the 'visual cycle'.<ref>PMID:19846785</ref>
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	Cellular retinaldehyde-binding protein (CRALBP) chaperones 11-cis-retinal to convert opsin receptor molecules into photosensitive retinoid pigments of the eye. We report a thermal secondary isomerase activity of CRALBP when bound to 9-cis-retinal. UV/vis and 1H NMR spectroscopy were used to characterize the product as 9,13-dicis-retinal. The X-ray structure of the CRALBP mutant R234W:9-cis-retinal complex at 1.9 A resolution revealed a niche in the binding pocket for 9-cis-aldehyde different from that reported for 11-cis-retinal. Combined computational, kinetic, and structural data lead us to propose an isomerization mechanism catalyzed by a network of buried waters. Our findings highlight a specific role of water molecules in both CRALBP-assisted specificity toward 9-cis-retinal and its thermal isomerase activity yielding 9,13-dicis-retinal. Kinetic data from two point mutants of CRALBP support an essential role of Glu202 as the initial proton donor in this isomerization reaction.

-	~~==About this Structure==~~	+	Human Cellular Retinaldehyde-Binding Protein Has Secondary Thermal 9-cis-Retinal Isomerase Activity.,Bolze CS, Helbling RE, Owen RL, Pearson AR, Pompidor G, Dworkowski F, Fuchs MR, Furrer J, Golczak M, Palczewski K, Cascella M, Stocker A J Am Chem Soc. 2013 Dec 20. PMID:24328211<ref>PMID:24328211</ref>
-	~~[[4cj6]] is a 1 chain structure~~. ~~Full crystallographic information is available from [http://oca~~.~~weizmann~~.~~ac.il~~/~~oca-bin/ocashort?id=4CJ6 OCA].~~	+

-	==~~Reference~~==	+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
-	~~<ref group="xtra">PMID:024328211</ref>~~<references ~~group="xtra"~~/><~~references~~/>	+	</div>
-	[[Category: Bolze, C S.]]	+	== References ==
-	[[Category: Cascella, M.]]	+	<references/>
-	[[Category: Dworkowski, F.]]	+	__TOC__
-	[[Category: Fuchs, M R.]]	+	</StructureSection>
-	[[Category: Furrer, J.]]	+	[[Category: Human]]
-	[[Category: Golczak, M.]]	+	[[Category: Bolze, C S]]
-	[[Category: Helbling, R E.]]	+	[[Category: Cascella, M]]
-	[[Category: Owen, R L.]]	+	[[Category: Dworkowski, F]]
-	[[Category: Palczewski, K.]]	+	[[Category: Fuchs, M R]]
-	[[Category: Pearson, A R.]]	+	[[Category: Furrer, J]]
-	[[Category: Pompidor, G.]]	+	[[Category: Golczak, M]]
-	[[Category: Stocker, A.]]	+	[[Category: Helbling, R E]]
		+	[[Category: Owen, R L]]
		+	[[Category: Palczewski, K]]
		+	[[Category: Pearson, A R]]
		+	[[Category: Pompidor, G]]
		+	[[Category: Stocker, A]]
	[[Category: 9-cis-retinal]]		[[Category: 9-cis-retinal]]
	[[Category: Isomerase]]		[[Category: Isomerase]]
	[[Category: Visual cycle]]		[[Category: Visual cycle]]

OCA at 10:04, 8 January 2014

OCA — Wed, 08 Jan 2014 10:04:01 GMT

←Older revision		Revision as of 10:04, 8 January 2014
Line 1:		Line 1:
-	~~'''Unreleased~~ structure~~'''~~	+	{{STRUCTURE_4cj6\| PDB=4cj6 \| SCENE= }}
		+	===Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal===
		+	{{ABSTRACT_PUBMED_24328211}}

-	~~The~~ entry ~~4cj6~~ is ~~ON HOLD~~	+	==Disease==
		+	[[http://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Defects in RLBP1 are a cause of retinitis pigmentosa autosomal recessive (ARRP) [MIM:[http://omim.org/entry/268000 268000]]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.<ref>PMID:9326942</ref> Defects in RLBP1 are the cause of Bothnia retinal dystrophy (BRD) [MIM:[http://omim.org/entry/607475 607475]]; also known as Vasterbotten dystrophy. Affected individuals show night blindness from early childhood with features consistent with retinitis punctata albescens and macular degeneration.<ref>PMID:10102298</ref> Defects in RLBP1 are the cause of rod-cone dystrophy Newfoundland (NFRCD) [MIM:[http://omim.org/entry/607476 607476]]. NFRCD is a retinal dystrophy reminiscent of retinitis punctata albescens but with a substantially lower age at onset and more-rapid and distinctive progression. Rod-cone dystrophies results from initial loss of rod photoreceptors, later followed by cone photoreceptors loss.<ref>PMID:11868161</ref> Defects in RLBP1 are a cause of retinitis punctata albescens (RPA) [MIM:[http://omim.org/entry/136880 136880]]. A rare form of stationary night blindness characterized by a delay in the regeneration of cone and rod photopigments.

-	~~Authors~~: ~~Bolze, C~~.S.~~, Helbling, R.E~~., ~~Owen, R~~.~~L., Pearson, A.R., Pompidor, G., Dworkowski, F., Fuchs, M.R., Furrer, J., Golczak, M., Palczewski, K., Cascella, M., Stocker, A~~.	+	==Function==
		+	[[http://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Soluble retinoid carrier essential the proper function of both rod and cone photoreceptors. Participates in the regeneration of active 11-cis-retinol and 11-cis-retinaldehyde, from the inactive 11-trans products of the rhodopsin photocycle and in the de novo synthesis of these retinoids from 11-trans metabolic precursors. The cycling of retinoids between photoreceptor and adjacent pigment epithelium cells is known as the 'visual cycle'.<ref>PMID:19846785</ref>

-	~~Description: Crystal~~ structure ~~of the complex of the Cellular Retinal Binding Protein Mutant R234W with~~ 9-cis-retinal	+	==About this Structure==
		+	[[4cj6]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4CJ6 OCA].
		+
		+	==Reference==
		+	<ref group="xtra">PMID:024328211</ref><references group="xtra"/><references/>
		+	[[Category: Bolze, C S.]]
		+	[[Category: Cascella, M.]]
		+	[[Category: Dworkowski, F.]]
		+	[[Category: Fuchs, M R.]]
		+	[[Category: Furrer, J.]]
		+	[[Category: Golczak, M.]]
		+	[[Category: Helbling, R E.]]
		+	[[Category: Owen, R L.]]
		+	[[Category: Palczewski, K.]]
		+	[[Category: Pearson, A R.]]
		+	[[Category: Pompidor, G.]]
		+	[[Category: Stocker, A.]]
		+	[[Category: 9-cis-retinal]]
		+	[[Category: Isomerase]]
		+	[[Category: Visual cycle]]

OCA: Protected "4cj6" [edit=sysop:move=sysop]

OCA — Wed, 25 Dec 2013 09:08:49 GMT

Protected "4cj6" [edit=sysop:move=sysop]

←Older revision

Revision as of 09:08, 25 December 2013

OCA: New page: '''Unreleased structure''' The entry 4cj6 is ON HOLD Authors: Bolze, C.S., Helbling, R.E., Owen, R.L., Pearson, A.R., Pompidor, G., Dworkowski, F., Fuchs, M.R., Furrer, J., Golczak, M.,...

OCA — Wed, 25 Dec 2013 09:08:48 GMT

New page: '''Unreleased structure''' The entry 4cj6 is ON HOLD Authors: Bolze, C.S., Helbling, R.E., Owen, R.L., Pearson, A.R., Pompidor, G., Dworkowski, F., Fuchs, M.R., Furrer, J., Golczak, M.,...

New page

'''Unreleased structure'''

The entry 4cj6 is ON HOLD

Authors: Bolze, C.S., Helbling, R.E., Owen, R.L., Pearson, A.R., Pompidor, G., Dworkowski, F., Fuchs, M.R., Furrer, J., Golczak, M., Palczewski, K., Cascella, M., Stocker, A.

Description: Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal