3qfr - Revision history

OCA at 11:44, 14 March 2024

OCA — Thu, 14 Mar 2024 11:44:07 GMT

←Older revision		Revision as of 11:44, 14 March 2024
Line 3:		Line 3:
	<StructureSection load='3qfr' size='340' side='right'caption='[[3qfr]], [[Resolution\|resolution]] 2.40Å' scene=''>		<StructureSection load='3qfr' size='340' side='right'caption='[[3qfr]], [[Resolution\|resolution]] 2.40Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[3qfr]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/~~Human Human~~]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3QFR FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[3qfr]] 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=3QFR OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3QFR FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=NAP:NADP+NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NAP</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]] 2.4Å</td></tr>
-	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat"><div style='overflow: auto; max-height: 3em;'>[[3qe2\|3qe2]], [[3qfc\|3qfc]], [[3qfs\|3qfs]], [[3qft\|3qft]]</div></td></tr>	+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=NAP:NADP+NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NAP</scene></td></tr>
-	~~<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">POR, CYPOR ([https://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'>[https://en.wikipedia.org/wiki/NADPH--hemoprotein_reductase NADPH--hemoprotein reductase], with EC number [https://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.6.2.4 1.6.2.4] </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=3qfr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3qfr OCA], [https://pdbe.org/3qfr PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3qfr RCSB], [https://www.ebi.ac.uk/pdbsum/3qfr PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3qfr 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=3qfr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3qfr OCA], [https://pdbe.org/3qfr PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3qfr RCSB], [https://www.ebi.ac.uk/pdbsum/3qfr PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3qfr ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
-	[[https://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] Defects in POR are the cause of Antley-Bixler syndrome with genital anomalies and disordered steroidogenesis (ABS1) [MIM:[https://omim.org/entry/201750 201750]]. A disease characterized by the association of Antley-Bixler syndrome with steroidogenesis defects and abnormal genitalia. Antley-Bixler syndrome is characterized by craniosynostosis, radiohumeral synostosis present from the perinatal period, midface hypoplasia, choanal stenosis or atresia, femoral bowing and multiple joint contractures.<ref>PMID:15264278</ref> <ref>PMID:15483095</ref> <ref>PMID:14758361</ref> Defects in POR are the cause of disordered steroidogenesis due to cytochrome P450 oxidoreductase deficiency (DISPORD) [MIM:[https://omim.org/entry/613571 613571]]. A disorder resulting in a rare variant of congenital adrenal hyperplasia, with apparent combined P450C17 and P450C21 deficiency and accumulation of steroid metabolites. Affected girls are born with ambiguous genitalia, but their circulating androgens are low and virilization does not progress. Conversely, affected boys are sometimes born undermasculinized. Boys and girls can present with bone malformations, in some cases resembling the pattern seen in patients with Antley-Bixler syndrome.<ref>PMID:14758361</ref> <ref>PMID:15220035</ref>	+	[https://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN] Defects in POR are the cause of Antley-Bixler syndrome with genital anomalies and disordered steroidogenesis (ABS1) [MIM:[https://omim.org/entry/201750 201750]. A disease characterized by the association of Antley-Bixler syndrome with steroidogenesis defects and abnormal genitalia. Antley-Bixler syndrome is characterized by craniosynostosis, radiohumeral synostosis present from the perinatal period, midface hypoplasia, choanal stenosis or atresia, femoral bowing and multiple joint contractures.<ref>PMID:15264278</ref> <ref>PMID:15483095</ref> <ref>PMID:14758361</ref> Defects in POR are the cause of disordered steroidogenesis due to cytochrome P450 oxidoreductase deficiency (DISPORD) [MIM:[https://omim.org/entry/613571 613571]. A disorder resulting in a rare variant of congenital adrenal hyperplasia, with apparent combined P450C17 and P450C21 deficiency and accumulation of steroid metabolites. Affected girls are born with ambiguous genitalia, but their circulating androgens are low and virilization does not progress. Conversely, affected boys are sometimes born undermasculinized. Boys and girls can present with bone malformations, in some cases resembling the pattern seen in patients with Antley-Bixler syndrome.<ref>PMID:14758361</ref> <ref>PMID:15220035</ref>
	== Function ==		== Function ==
-	[[https://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] This enzyme is required for electron transfer from NADP to cytochrome P450 in microsomes. It can also provide electron transfer to heme oxygenase and cytochrome B5.	+	[https://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN] This enzyme is required for electron transfer from NADP to cytochrome P450 in microsomes. It can also provide electron transfer to heme oxygenase and cytochrome B5.
-	~~<div style="background-color:#fffaf0;">~~	+
-	~~== Publication Abstract from PubMed ==~~	+
-	NADPH-cytochrome P450 oxidoreductase (CYPOR) is essential for electron donation to microsomal cytochrome P450-mediated monooxygenation in such diverse physiological processes as drug metabolism (approximately 85-90% of therapeutic drugs), steroid biosynthesis, and bioactive metabolite production (vitamin D and retinoic acid metabolites). Expressed by a single gene, CYPOR's role with these multiple redox partners renders it a model for understanding protein-protein interactions at the structural level. Polymorphisms in human CYPOR have been shown to lead to defects in bone development and steroidogenesis, resulting in sexual dimorphisms, the severity of which differs significantly depending on the degree of CYPOR impairment. The atomic structure of human CYPOR is presented, with structures of two naturally occurring missense mutations, V492E and R457H. The overall structures of these CYPOR variants are similar to wild type. However, in both variants, local disruption of H bonding and salt bridging, involving the FAD pyrophosphate moiety, leads to weaker FAD binding, unstable protein, and loss of catalytic activity, which can be rescued by cofactor addition. The modes of polypeptide unfolding in these two variants differ significantly, as revealed by limited trypsin digestion: V492E is less stable but unfolds locally and gradually, whereas R457H is more stable but unfolds globally. FAD addition to either variant prevents trypsin digestion, supporting the role of the cofactor in conferring stability to CYPOR structure. Thus, CYPOR dysfunction in patients harboring these particular mutations may possibly be prevented by riboflavin therapy in utero, if predicted prenatally, or rescued postnatally in less severe cases.	+
-		+
-	Structural basis for human NADPH-cytochrome P450 oxidoreductase deficiency.,Xia C, Panda SP, Marohnic CC, Martasek P, Masters BS, Kim JJ Proc Natl Acad Sci U S A. 2011 Aug 16;108(33):13486-91. Epub 2011 Aug 1. PMID:21808038<ref>PMID:21808038</ref>	+
-		+
-	~~From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>~~	+
-	~~</div>~~	+
-	~~<div class="pdbe-citations 3qfr" style="background-color:#fffaf0;"></div>~~	+

	==See Also==		==See Also==
Line 30:		Line 19:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Human~~]]	+	[[Category: Homo sapiens]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	~~[[Category: NADPH--hemoprotein reductase]]~~	+	[[Category: Kim J-JP]]
-	[[Category: Kim, J ~~J.P~~]]	+	[[Category: Marohnic C]]
-	[[Category: Marohnic, C]]	+	[[Category: Masters BS]]
-	[[Category: Masters~~, B S~~]]	+	[[Category: Panda SP]]
-	[[Category: Panda~~, S P~~]]	+	[[Category: Xia C]]
-	[[Category: Xia, C]]	+
-	~~[[Category: Antley-bixler syndrome]]~~	+
-	~~[[Category: Fad]]~~	+
-	~~[[Category: Flavoprotein]]~~	+
-	~~[[Category: Fmn]]~~	+
-	~~[[Category: Nadph]]~~	+
-	~~[[Category: Nadph-cytochrome p450 reductase]]~~	+
-	~~[[Category: Oxidoreductase~~]]	+

OCA at 06:07, 8 June 2022

OCA — Wed, 08 Jun 2022 06:07:13 GMT

←Older revision		Revision as of 06:07, 8 June 2022
Line 1:		Line 1:

	==Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)==		==Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)==
-	<StructureSection load='3qfr' size='340' side='right' caption='[[3qfr]], [[Resolution\|resolution]] 2.40Å' scene=''>	+	<StructureSection load='3qfr' size='340' side='right'caption='[[3qfr]], [[Resolution\|resolution]] 2.40Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[3qfr]] 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=3QFR OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://~~oca~~.~~weizmann.ac.il/oca-docs~~/fgij/fg.htm?mol=3QFR FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[3qfr]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3QFR FirstGlance]. <br>
-	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=NAP:NADP+NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NAP</scene></td></tr>	+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=NAP:NADP+NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NAP</scene></td></tr>
-	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[3qe2\|3qe2]], [[3qfc\|3qfc]], [[3qfs\|3qfs]], [[3qft\|3qft]]</td></tr>	+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat"><div style='overflow: auto; max-height: 3em;'>[[3qe2\|3qe2]], [[3qfc\|3qfc]], [[3qfs\|3qfs]], [[3qft\|3qft]]</div></td></tr>
-	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">POR, CYPOR ([~~http~~://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>	+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">POR, CYPOR ([https://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/NADPH--hemoprotein_reductase NADPH--hemoprotein reductase], with EC number [~~http~~://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.6.2.4 1.6.2.4] </span></td></tr>	+	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[https://en.wikipedia.org/wiki/NADPH--hemoprotein_reductase NADPH--hemoprotein reductase], with EC number [https://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.6.2.4 1.6.2.4] </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=3qfr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3qfr OCA], [~~http~~://pdbe.org/3qfr PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=3qfr RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/3qfr PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=3qfr 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=3qfr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3qfr OCA], [https://pdbe.org/3qfr PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3qfr RCSB], [https://www.ebi.ac.uk/pdbsum/3qfr PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3qfr ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
-	[[~~http~~://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] Defects in POR are the cause of Antley-Bixler syndrome with genital anomalies and disordered steroidogenesis (ABS1) [MIM:[~~http~~://omim.org/entry/201750 201750]]. A disease characterized by the association of Antley-Bixler syndrome with steroidogenesis defects and abnormal genitalia. Antley-Bixler syndrome is characterized by craniosynostosis, radiohumeral synostosis present from the perinatal period, midface hypoplasia, choanal stenosis or atresia, femoral bowing and multiple joint contractures.<ref>PMID:15264278</ref> <ref>PMID:15483095</ref> <ref>PMID:14758361</ref> Defects in POR are the cause of disordered steroidogenesis due to cytochrome P450 oxidoreductase deficiency (DISPORD) [MIM:[~~http~~://omim.org/entry/613571 613571]]. A disorder resulting in a rare variant of congenital adrenal hyperplasia, with apparent combined P450C17 and P450C21 deficiency and accumulation of steroid metabolites. Affected girls are born with ambiguous genitalia, but their circulating androgens are low and virilization does not progress. Conversely, affected boys are sometimes born undermasculinized. Boys and girls can present with bone malformations, in some cases resembling the pattern seen in patients with Antley-Bixler syndrome.<ref>PMID:14758361</ref> <ref>PMID:15220035</ref>	+	[[https://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] Defects in POR are the cause of Antley-Bixler syndrome with genital anomalies and disordered steroidogenesis (ABS1) [MIM:[https://omim.org/entry/201750 201750]]. A disease characterized by the association of Antley-Bixler syndrome with steroidogenesis defects and abnormal genitalia. Antley-Bixler syndrome is characterized by craniosynostosis, radiohumeral synostosis present from the perinatal period, midface hypoplasia, choanal stenosis or atresia, femoral bowing and multiple joint contractures.<ref>PMID:15264278</ref> <ref>PMID:15483095</ref> <ref>PMID:14758361</ref> Defects in POR are the cause of disordered steroidogenesis due to cytochrome P450 oxidoreductase deficiency (DISPORD) [MIM:[https://omim.org/entry/613571 613571]]. A disorder resulting in a rare variant of congenital adrenal hyperplasia, with apparent combined P450C17 and P450C21 deficiency and accumulation of steroid metabolites. Affected girls are born with ambiguous genitalia, but their circulating androgens are low and virilization does not progress. Conversely, affected boys are sometimes born undermasculinized. Boys and girls can present with bone malformations, in some cases resembling the pattern seen in patients with Antley-Bixler syndrome.<ref>PMID:14758361</ref> <ref>PMID:15220035</ref>
	== Function ==		== Function ==
-	[[~~http~~://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] This enzyme is required for electron transfer from NADP to cytochrome P450 in microsomes. It can also provide electron transfer to heme oxygenase and cytochrome B5.	+	[[https://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] This enzyme is required for electron transfer from NADP to cytochrome P450 in microsomes. It can also provide electron transfer to heme oxygenase and cytochrome B5.
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
Line 31:		Line 31:
	</StructureSection>		</StructureSection>
	[[Category: Human]]		[[Category: Human]]
		+	[[Category: Large Structures]]
	[[Category: NADPH--hemoprotein reductase]]		[[Category: NADPH--hemoprotein reductase]]
	[[Category: Kim, J J.P]]		[[Category: Kim, J J.P]]

OCA at 08:49, 5 August 2016

OCA — Fri, 05 Aug 2016 08:49:27 GMT

←Older revision		Revision as of 08:49, 5 August 2016
Line 1:		Line 1:
		+
	==Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)==		==Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)==
	<StructureSection load='3qfr' size='340' side='right' caption='[[3qfr]], [[Resolution\|resolution]] 2.40Å' scene=''>		<StructureSection load='3qfr' size='340' side='right' caption='[[3qfr]], [[Resolution\|resolution]] 2.40Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[3qfr]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/~~Homo_sapiens Homo sapiens~~]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3QFR FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[3qfr]] 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=3QFR OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3QFR FirstGlance]. <br>
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=NAP:NADP+NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NAP</scene></td></tr>		</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=NAP:NADP+NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NAP</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[3qe2\|3qe2]], [[3qfc\|3qfc]], [[3qfs\|3qfs]], [[3qft\|3qft]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[3qe2\|3qe2]], [[3qfc\|3qfc]], [[3qfs\|3qfs]], [[3qft\|3qft]]</td></tr>
-	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">POR, CYPOR ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 ~~Homo sapiens~~])</td></tr>	+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">POR, CYPOR ([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/NADPH--hemoprotein_reductase NADPH--hemoprotein reductase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.6.2.4 1.6.2.4] </span></td></tr>		<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/NADPH--hemoprotein_reductase NADPH--hemoprotein reductase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.6.2.4 1.6.2.4] </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=3qfr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3qfr OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3qfr RCSB], [http://www.ebi.ac.uk/pdbsum/3qfr 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=3qfr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3qfr OCA], [http://pdbe.org/3qfr PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=3qfr RCSB], [http://www.ebi.ac.uk/pdbsum/3qfr PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=3qfr ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 21:		Line 22:
	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 3qfr" style="background-color:#fffaf0;"></div>

	==See Also==		==See Also==
Line 28:		Line 30:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Homo sapiens~~]]	+	[[Category: Human]]
	[[Category: NADPH--hemoprotein reductase]]		[[Category: NADPH--hemoprotein reductase]]
	[[Category: Kim, J J.P]]		[[Category: Kim, J J.P]]

OCA at 10:52, 19 December 2014

OCA — Fri, 19 Dec 2014 10:52:28 GMT

←Older revision		Revision as of 10:52, 19 December 2014
Line 1:		Line 1:
-	~~{{STRUCTURE_3qfr\| PDB=3qfr \| SCENE= }}~~	+	==Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)==
-	===Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)===	+	<StructureSection load='3qfr' size='340' side='right' caption='[[3qfr]], [[Resolution\|resolution]] 2.40Å' scene=''>
-	~~{{ABSTRACT_PUBMED_21808038}}~~	+	== Structural highlights ==
		+	<table><tr><td colspan='2'>[[3qfr]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3QFR FirstGlance]. <br>
		+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=NAP:NADP+NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NAP</scene></td></tr>
		+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[3qe2\|3qe2]], [[3qfc\|3qfc]], [[3qfs\|3qfs]], [[3qft\|3qft]]</td></tr>
		+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">POR, CYPOR ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
		+	<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/NADPH--hemoprotein_reductase NADPH--hemoprotein reductase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.6.2.4 1.6.2.4] </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=3qfr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3qfr OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3qfr RCSB], [http://www.ebi.ac.uk/pdbsum/3qfr PDBsum]</span></td></tr>
		+	</table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] Defects in POR are the cause of Antley-Bixler syndrome with genital anomalies and disordered steroidogenesis (ABS1) [MIM:[http://omim.org/entry/201750 201750]]. A disease characterized by the association of Antley-Bixler syndrome with steroidogenesis defects and abnormal genitalia. Antley-Bixler syndrome is characterized by craniosynostosis, radiohumeral synostosis present from the perinatal period, midface hypoplasia, choanal stenosis or atresia, femoral bowing and multiple joint contractures.<ref>PMID:15264278</ref> <ref>PMID:15483095</ref> <ref>PMID:14758361</ref> Defects in POR are the cause of disordered steroidogenesis due to cytochrome P450 oxidoreductase deficiency (DISPORD) [MIM:[http://omim.org/entry/613571 613571]]. A disorder resulting in a rare variant of congenital adrenal hyperplasia, with apparent combined P450C17 and P450C21 deficiency and accumulation of steroid metabolites. Affected girls are born with ambiguous genitalia, but their circulating androgens are low and virilization does not progress. Conversely, affected boys are sometimes born undermasculinized. Boys and girls can present with bone malformations, in some cases resembling the pattern seen in patients with Antley-Bixler syndrome.<ref>PMID:14758361</ref> <ref>PMID:15220035</ref>
		+	== Function ==
		+	[[http://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] This enzyme is required for electron transfer from NADP to cytochrome P450 in microsomes. It can also provide electron transfer to heme oxygenase and cytochrome B5.
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	NADPH-cytochrome P450 oxidoreductase (CYPOR) is essential for electron donation to microsomal cytochrome P450-mediated monooxygenation in such diverse physiological processes as drug metabolism (approximately 85-90% of therapeutic drugs), steroid biosynthesis, and bioactive metabolite production (vitamin D and retinoic acid metabolites). Expressed by a single gene, CYPOR's role with these multiple redox partners renders it a model for understanding protein-protein interactions at the structural level. Polymorphisms in human CYPOR have been shown to lead to defects in bone development and steroidogenesis, resulting in sexual dimorphisms, the severity of which differs significantly depending on the degree of CYPOR impairment. The atomic structure of human CYPOR is presented, with structures of two naturally occurring missense mutations, V492E and R457H. The overall structures of these CYPOR variants are similar to wild type. However, in both variants, local disruption of H bonding and salt bridging, involving the FAD pyrophosphate moiety, leads to weaker FAD binding, unstable protein, and loss of catalytic activity, which can be rescued by cofactor addition. The modes of polypeptide unfolding in these two variants differ significantly, as revealed by limited trypsin digestion: V492E is less stable but unfolds locally and gradually, whereas R457H is more stable but unfolds globally. FAD addition to either variant prevents trypsin digestion, supporting the role of the cofactor in conferring stability to CYPOR structure. Thus, CYPOR dysfunction in patients harboring these particular mutations may possibly be prevented by riboflavin therapy in utero, if predicted prenatally, or rescued postnatally in less severe cases.

-	~~==Disease==~~	+	Structural basis for human NADPH-cytochrome P450 oxidoreductase deficiency.,Xia C, Panda SP, Marohnic CC, Martasek P, Masters BS, Kim JJ Proc Natl Acad Sci U S A. 2011 Aug 16;108(33):13486-91. Epub 2011 Aug 1. PMID:21808038<ref>PMID:21808038</ref>
-	~~[[http://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] Defects in POR are the cause of Antley~~-~~Bixler syndrome with genital anomalies and disordered steroidogenesis (ABS1) [MIM:[http://omim~~.org/entry/201750 201750]]. A disease characterized by the association of Antley-Bixler syndrome with steroidogenesis defects and abnormal genitalia. Antley-Bixler syndrome is characterized by craniosynostosis, ~~radiohumeral synostosis present from the perinatal period~~, ~~midface hypoplasia~~, ~~choanal stenosis or atresia~~, ~~femoral bowing and multiple joint contractures~~.~~<ref>PMID:15264278</ref><ref>PMID:15483095</ref><ref>PMID:14758361</ref> Defects in POR are the cause of disordered steroidogenesis due to cytochrome P450 oxidoreductase deficiency~~ (~~DISPORD~~) ~~[MIM~~:~~[http://omim~~.~~org/entry/613571 613571]]~~. A disorder resulting in a rare variant of congenital adrenal hyperplasia, with apparent combined P450C17 and P450C21 deficiency and accumulation of steroid metabolites. Affected girls are born with ambiguous genitalia, but their circulating androgens are low and virilization does not progress. Conversely, affected boys are sometimes born undermasculinized. Boys and girls can present with bone malformations, in some cases resembling the pattern seen in patients with Antley-Bixler syndrome.<ref>PMID:~~14758361</ref>~~<ref>PMID:~~15220035~~</ref>	+

-	~~==Function==~~	+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
-	~~[[http:~~/~~/www~~.~~uniprot~~.~~org/uniprot/NCPR_HUMAN NCPR_HUMAN]] This enzyme is required for electron transfer from NADP to cytochrome P450 in microsomes. It can also provide electron transfer to heme oxygenase and cytochrome B5~~.	+	</div>
-		+
-	~~==About this Structure==~~	+
-	~~[[3qfr]] is a 2 chain structure with sequence from [http:~~/~~/en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA].~~	+

	==See Also==		==See Also==
	*[[NADPH-Cytochrome P450 Reductase\|NADPH-Cytochrome P450 Reductase]]		*[[NADPH-Cytochrome P450 Reductase\|NADPH-Cytochrome P450 Reductase]]
-		+	== References ==
-	==~~Reference~~==	+	<references/>
-	~~<ref group="xtra">PMID:021808038</ref>~~<references ~~group="xtra"~~/><~~references~~/>	+	__TOC__
		+	</StructureSection>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: NADPH--hemoprotein reductase]]		[[Category: NADPH--hemoprotein reductase]]
-	[[Category: Kim, J J.P.]]	+	[[Category: Kim, J J.P]]
-	[[Category: Marohnic, C.]]	+	[[Category: Marohnic, C]]
-	[[Category: Masters, B S.]]	+	[[Category: Masters, B S]]
-	[[Category: Panda, S P.]]	+	[[Category: Panda, S P]]
-	[[Category: Xia, C.]]	+	[[Category: Xia, C]]
	[[Category: Antley-bixler syndrome]]		[[Category: Antley-bixler syndrome]]
	[[Category: Fad]]		[[Category: Fad]]

OCA at 18:55, 24 March 2013

OCA — Sun, 24 Mar 2013 18:55:40 GMT

←Older revision		Revision as of 18:55, 24 March 2013
Line 1:		Line 1:
-	[[Image:3qfr.png\|left\|200px]]
-
	{{STRUCTURE_3qfr\| PDB=3qfr \| SCENE= }}		{{STRUCTURE_3qfr\| PDB=3qfr \| SCENE= }}
-
	===Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)===		===Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)===
		+	{{ABSTRACT_PUBMED_21808038}}

-	~~{{ABSTRACT_PUBMED_21808038}}~~	+	==Disease==
		+	[[http://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] Defects in POR are the cause of Antley-Bixler syndrome with genital anomalies and disordered steroidogenesis (ABS1) [MIM:[http://omim.org/entry/201750 201750]]. A disease characterized by the association of Antley-Bixler syndrome with steroidogenesis defects and abnormal genitalia. Antley-Bixler syndrome is characterized by craniosynostosis, radiohumeral synostosis present from the perinatal period, midface hypoplasia, choanal stenosis or atresia, femoral bowing and multiple joint contractures.<ref>PMID:15264278</ref><ref>PMID:15483095</ref><ref>PMID:14758361</ref> Defects in POR are the cause of disordered steroidogenesis due to cytochrome P450 oxidoreductase deficiency (DISPORD) [MIM:[http://omim.org/entry/613571 613571]]. A disorder resulting in a rare variant of congenital adrenal hyperplasia, with apparent combined P450C17 and P450C21 deficiency and accumulation of steroid metabolites. Affected girls are born with ambiguous genitalia, but their circulating androgens are low and virilization does not progress. Conversely, affected boys are sometimes born undermasculinized. Boys and girls can present with bone malformations, in some cases resembling the pattern seen in patients with Antley-Bixler syndrome.<ref>PMID:14758361</ref><ref>PMID:15220035</ref>
		+
		+	==Function==
		+	[[http://www.uniprot.org/uniprot/NCPR_HUMAN NCPR_HUMAN]] This enzyme is required for electron transfer from NADP to cytochrome P450 in microsomes. It can also provide electron transfer to heme oxygenase and cytochrome B5.

	==About this Structure==		==About this Structure==
Line 14:		Line 16:

	==Reference==		==Reference==
-	<ref group="xtra">PMID:021808038</ref><references group="xtra"/>	+	<ref group="xtra">PMID:021808038</ref><references group="xtra"/><references/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: NADPH--hemoprotein reductase]]		[[Category: NADPH--hemoprotein reductase]]

OCA at 19:24, 20 October 2012

OCA — Sat, 20 Oct 2012 19:24:58 GMT

←Older revision		Revision as of 19:24, 20 October 2012
Line 8:		Line 8:

	==About this Structure==		==About this Structure==
-	[[3qfr]] is a 2 chain structure ~~of [[NADPH-Cytochrome P450 Reductase]]~~ with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA].	+	[[3qfr]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA].

	==See Also==		==See Also==

OCA at 07:17, 27 July 2012

OCA — Fri, 27 Jul 2012 07:17:05 GMT

←Older revision		Revision as of 07:17, 27 July 2012
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	[[Image:3qfr.png\|left\|200px]]		[[Image:3qfr.png\|left\|200px]]

-	<!--
-	The line below this paragraph, containing "STRUCTURE_3qfr", creates the "Structure Box" on the page.
-	You may change the PDB parameter (which sets the PDB file loaded into the applet)
-	or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
-	or leave the SCENE parameter empty for the default display.
-	-->
	{{STRUCTURE_3qfr\| PDB=3qfr \| SCENE= }}		{{STRUCTURE_3qfr\| PDB=3qfr \| SCENE= }}

	===Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)===		===Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)===

-
-	<!--
-	The line below this paragraph, {{ABSTRACT_PUBMED_21808038}}, adds the Publication Abstract to the page
-	(as it appears on PubMed at http://www.pubmed.gov), where 21808038 is the PubMed ID number.
-	-->
	{{ABSTRACT_PUBMED_21808038}}		{{ABSTRACT_PUBMED_21808038}}

	==About this Structure==		==About this Structure==
-	[[3qfr]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA].	+	[[3qfr]] is a 2 chain structure of [[NADPH-Cytochrome P450 Reductase]] with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA].
		+
		+	==See Also==
		+	*[[NADPH-Cytochrome P450 Reductase\|NADPH-Cytochrome P450 Reductase]]

	==Reference==		==Reference==

OCA at 06:20, 24 August 2011

OCA — Wed, 24 Aug 2011 06:20:10 GMT

←Older revision		Revision as of 06:20, 24 August 2011
Line 1:		Line 1:
-	[[Image:3qfr.~~jpg~~\|left\|200px]]	+	[[Image:3qfr.png\|left\|200px]]

	<!--		<!--
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	===Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)===		===Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)===

		+
		+	<!--
		+	The line below this paragraph, {{ABSTRACT_PUBMED_21808038}}, adds the Publication Abstract to the page
		+	(as it appears on PubMed at http://www.pubmed.gov), where 21808038 is the PubMed ID number.
		+	-->
		+	{{ABSTRACT_PUBMED_21808038}}

	==About this Structure==		==About this Structure==
	[[3qfr]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA].		[[3qfr]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA].
		+
		+	==Reference==
		+	<ref group="xtra">PMID:021808038</ref><references group="xtra"/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: NADPH--hemoprotein reductase]]		[[Category: NADPH--hemoprotein reductase]]

OCA at 08:58, 3 August 2011

OCA — Wed, 03 Aug 2011 08:58:07 GMT

←Older revision		Revision as of 08:58, 3 August 2011
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-	~~'''Unreleased structure'''~~	+	[[Image:3qfr.jpg\|left\|200px]]

-	The ~~entry 3qfr~~ is ~~ON HOLD~~ ~~until Paper Publication~~	+	<!--
		+	The line below this paragraph, containing "STRUCTURE_3qfr", creates the "Structure Box" on the page.
		+	You may change the PDB parameter (which sets the PDB file loaded into the applet)
		+	or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
		+	or leave the SCENE parameter empty for the default display.
		+	-->
		+	{{STRUCTURE_3qfr\| PDB=3qfr \| SCENE= }}

-	~~Authors: Xia, C., Marohnic, C., Panda, S.P., Masters, B.S., Kim, J.~~-~~J.P.~~	+	===Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)===

-	~~Description: Crystal~~ Structure ~~of Human~~ NADPH-~~Cytochrome P450 Reductase (R457H Mutant)~~	+
		+	==About this Structure==
		+	[[3qfr]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3QFR OCA].
		+	[[Category: Homo sapiens]]
		+	[[Category: NADPH--hemoprotein reductase]]
		+	[[Category: Kim, J J.P.]]
		+	[[Category: Marohnic, C.]]
		+	[[Category: Masters, B S.]]
		+	[[Category: Panda, S P.]]
		+	[[Category: Xia, C.]]
		+	[[Category: Antley-bixler syndrome]]
		+	[[Category: Fad]]
		+	[[Category: Flavoprotein]]
		+	[[Category: Fmn]]
		+	[[Category: Nadph]]
		+	[[Category: Nadph-cytochrome p450 reductase]]
		+	[[Category: Oxidoreductase]]

OCA at 06:38, 2 March 2011

OCA — Wed, 02 Mar 2011 06:38:51 GMT

←Older revision		Revision as of 06:38, 2 March 2011
Line 1:		Line 1:
	'''Unreleased structure'''		'''Unreleased structure'''

-	The entry 3qfr is ON HOLD	+	The entry 3qfr is ON HOLD until Paper Publication

	Authors: Xia, C., Marohnic, C., Panda, S.P., Masters, B.S., Kim, J.-J.P.		Authors: Xia, C., Marohnic, C., Panda, S.P., Masters, B.S., Kim, J.-J.P.

-	Description: Crystal Structure of Human NADPH-Cytochrome P450 Reductase (~~R454H~~ Mutant)	+	Description: Crystal Structure of Human NADPH-Cytochrome P450 Reductase (R457H Mutant)