3myj - Revision history

OCA at 09:04, 6 September 2023

OCA — Wed, 06 Sep 2023 09:04:29 GMT

OCA at 08:20, 8 November 2017

OCA — Wed, 08 Nov 2017 08:20:17 GMT

←Older revision		Revision as of 08:20, 8 November 2017
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	</div>		</div>
	<div class="pdbe-citations 3myj" style="background-color:#fffaf0;"></div>		<div class="pdbe-citations 3myj" style="background-color:#fffaf0;"></div>
-
-	==See Also==
-	*[[Beta-2 microglobulin\|Beta-2 microglobulin]]
-	*[[Major histocompatibility complex\|Major histocompatibility complex]]
	== References ==		== References ==
	<references/>		<references/>

OCA at 14:17, 27 April 2017

OCA — Thu, 27 Apr 2017 14:17:27 GMT

←Older revision		Revision as of 14:17, 27 April 2017
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		+
	==Human Class I MHC HLA-A2 in complex with the WT-1 (126-134) (R1Y) peptide variant.==		==Human Class I MHC HLA-A2 in complex with the WT-1 (126-134) (R1Y) peptide variant.==
	<StructureSection load='3myj' size='340' side='right' caption='[[3myj]], [[Resolution\|resolution]] 1.89Å' scene=''>		<StructureSection load='3myj' size='340' side='right' caption='[[3myj]], [[Resolution\|resolution]] 1.89Å' scene=''>
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	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[3hpj\|3hpj]], [[1tvb\|1tvb]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[3hpj\|3hpj]], [[1tvb\|1tvb]]</td></tr>
	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">HLA-A, HLAA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN]), B2M, CDABP0092, HDCMA22P ([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">HLA-A, HLAA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN]), B2M, CDABP0092, HDCMA22P ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
-	<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3myj FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3myj OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3myj RCSB], [http://www.ebi.ac.uk/pdbsum/3myj 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=3myj FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3myj OCA], [http://pdbe.org/3myj PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=3myj RCSB], [http://www.ebi.ac.uk/pdbsum/3myj PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=3myj ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
Line 20:		Line 21:
	<text>to colour the structure by Evolutionary Conservation</text>		<text>to colour the structure by Evolutionary Conservation</text>
	</jmolCheckbox>		</jmolCheckbox>
-	</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary\|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/~~chain_selection~~.php?pdb_ID=~~2ata~~ ConSurf].	+	</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary\|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=3myj ConSurf].
	<div style="clear:both"></div>		<div style="clear:both"></div>
	<div style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
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	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 3myj" style="background-color:#fffaf0;"></div>

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

OCA at 08:06, 22 July 2015

OCA — Wed, 22 Jul 2015 08:06:25 GMT

←Older revision		Revision as of 08:06, 22 July 2015
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	<StructureSection load='3myj' size='340' side='right' caption='[[3myj]], [[Resolution\|resolution]] 1.89Å' scene=''>		<StructureSection load='3myj' size='340' side='right' caption='[[3myj]], [[Resolution\|resolution]] 1.89Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[3myj]] is a 6 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=3MYJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3MYJ FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[3myj]] is a 6 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=3MYJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3MYJ FirstGlance]. <br>
	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></td></tr>		</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></td></tr>
	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[3hpj\|3hpj]], [[1tvb\|1tvb]]</td></tr>		<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[3hpj\|3hpj]], [[1tvb\|1tvb]]</td></tr>
-	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">HLA-A, HLAA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 ~~Homo sapiens~~]), B2M, CDABP0092, HDCMA22P ([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">HLA-A, HLAA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN]), B2M, CDABP0092, HDCMA22P ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
	<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3myj FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3myj OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3myj RCSB], [http://www.ebi.ac.uk/pdbsum/3myj 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=3myj FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3myj OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3myj RCSB], [http://www.ebi.ac.uk/pdbsum/3myj PDBsum]</span></td></tr>
	</table>		</table>
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	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Homo sapiens~~]]	+	[[Category: Human]]
	[[Category: Baker, B M]]		[[Category: Baker, B M]]
	[[Category: Borbulevych, O Y]]		[[Category: Borbulevych, O Y]]

OCA at 17:01, 18 December 2014

OCA — Thu, 18 Dec 2014 17:01:36 GMT

←Older revision		Revision as of 17:01, 18 December 2014
Line 1:		Line 1:
-	~~{{STRUCTURE_3myj\| PDB=3myj \| SCENE= }}~~	+	==Human Class I MHC HLA-A2 in complex with the WT-1 (126-134) (R1Y) peptide variant.==
-	===Human Class I MHC HLA-A2 in complex with the WT-1 (126-134) (R1Y) peptide variant.===	+	<StructureSection load='3myj' size='340' side='right' caption='[[3myj]], [[Resolution\|resolution]] 1.89Å' scene=''>
-	~~{{ABSTRACT_PUBMED_20619457}}~~	+	== Structural highlights ==
		+	<table><tr><td colspan='2'>[[3myj]] is a 6 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=3MYJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3MYJ FirstGlance]. <br>
		+	</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></td></tr>
		+	<tr id='related'><td class="sblockLbl"><b>[[Related_structure\|Related:]]</b></td><td class="sblockDat">[[3hpj\|3hpj]], [[1tvb\|1tvb]]</td></tr>
		+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">HLA-A, HLAA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens]), B2M, CDABP0092, HDCMA22P ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</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=3myj FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3myj OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3myj RCSB], [http://www.ebi.ac.uk/pdbsum/3myj PDBsum]</span></td></tr>
		+	</table>
		+	== Disease ==
		+	[[http://www.uniprot.org/uniprot/WT1_HUMAN WT1_HUMAN]] Defects in WT1 are the cause of Frasier syndrome (FS) [MIM:[http://omim.org/entry/136680 136680]]. FS is characterized by a slowly progressing nephropathy leading to renal failure in adolescence or early adulthood, male pseudohermaphroditism, and no Wilms tumor. As for histological findings of the kidneys, focal glomerular sclerosis is often observed. There is phenotypic overlap with Denys-Drash syndrome. Inheritance is autosomal dominant.<ref>PMID:10571943</ref> Defects in WT1 are the cause of Wilms tumor 1 (WT1) [MIM:[http://omim.org/entry/194070 194070]]. WT is an embryonal malignancy of the kidney that affects approximately 1 in 10'000 infants and young children. It occurs both in sporadic and hereditary forms.<ref>PMID:1317572</ref> <ref>PMID:9108089</ref> <ref>PMID:9529364</ref> <ref>PMID:15150775</ref> Defects in WT1 are the cause of Denys-Drash syndrome (DDS) [MIM:[http://omim.org/entry/194080 194080]]. DDS is a typical nephropathy characterized by diffuse mesangial sclerosis, genital abnormalities, and/or Wilms tumor. There is phenotypic overlap with WAGR syndrome and Frasier syndrome. Inheritance is autosomal dominant, but most cases are sporadic.<ref>PMID:9529364</ref> <ref>PMID:1655284</ref> <ref>PMID:1302008</ref> <ref>PMID:1338906</ref> <ref>PMID:8388765</ref> <ref>PMID:8111391</ref> <ref>PMID:8295405</ref> <ref>PMID:8411073</ref> <ref>PMID:8112732</ref> <ref>PMID:8741319</ref> <ref>PMID:8956030</ref> <ref>PMID:9475094</ref> <ref>PMID:10738002</ref> <ref>PMID:11182928</ref> <ref>PMID:10799199</ref> <ref>PMID:11519891</ref> <ref>PMID:15349765</ref> Defects in WT1 are the cause of nephrotic syndrome type 4 (NPHS4) [MIM:[http://omim.org/entry/256370 256370]]. A renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. Most patients with NPHS4 show diffuse mesangial sclerosis on renal biopsy, which is a pathologic entity characterized by mesangial matrix expansion with no mesangial hypercellularity, hypertrophy of the podocytes, vacuolized podocytes, thickened basement membranes, and diminished patency of the capillary lumen.<ref>PMID:9529364</ref> <ref>PMID:11182928</ref> <ref>PMID:9607189</ref> <ref>PMID:15253707</ref> Defects in WT1 are a cause of Meacham syndrome (MEACHS) [MIM:[http://omim.org/entry/608978 608978]]. Meacham syndrome is a rare sporadically occurring multiple malformation syndrome characterized by male pseudohermaphroditism with abnormal internal female genitalia comprising a uterus and double or septate vagina, complex congenital heart defect and diaphragmatic abnormalities.<ref>PMID:17853480</ref> Note=A chromosomal aberration involving WT1 may be a cause of desmoplastic small round cell tumor (DSRCT). Translocation t(11;22)(p13;q12) with EWSR1. Defects in WT1 may be a cause of mesothelioma malignant (MESOM) [MIM:[http://omim.org/entry/156240 156240]]. An aggressive neoplasm of the serosal lining of the chest. It appears as broad sheets of cells, with some regions containing spindle-shaped, sarcoma-like cells and other regions showing adenomatous patterns. Pleural mesotheliomas have been linked to exposure to asbestos.<ref>PMID:8401592</ref> [[http://www.uniprot.org/uniprot/B2MG_HUMAN B2MG_HUMAN]] Defects in B2M are the cause of hypercatabolic hypoproteinemia (HYCATHYP) [MIM:[http://omim.org/entry/241600 241600]]. Affected individuals show marked reduction in serum concentrations of immunoglobulin and albumin, probably due to rapid degradation.<ref>PMID:16549777</ref> Note=Beta-2-microglobulin may adopt the fibrillar configuration of amyloid in certain pathologic states. The capacity to assemble into amyloid fibrils is concentration dependent. Persistently high beta(2)-microglobulin serum levels lead to amyloidosis in patients on long-term hemodialysis.<ref>PMID:3532124</ref> <ref>PMID:1336137</ref> <ref>PMID:7554280</ref> <ref>PMID:4586824</ref> <ref>PMID:8084451</ref> <ref>PMID:12119416</ref> <ref>PMID:12796775</ref> <ref>PMID:16901902</ref> <ref>PMID:16491088</ref> <ref>PMID:17646174</ref> <ref>PMID:18835253</ref> <ref>PMID:18395224</ref> <ref>PMID:19284997</ref>
		+	== Function ==
		+	[[http://www.uniprot.org/uniprot/1A02_HUMAN 1A02_HUMAN]] Involved in the presentation of foreign antigens to the immune system. [[http://www.uniprot.org/uniprot/WT1_HUMAN WT1_HUMAN]] Transcription factor that plays an important role in cellular development and cell survival. Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. Recognizes and binds to the DNA sequence 5'-CGCCCCCGC-3'. It has a tumor suppressor as well as an oncogenic role in tumor formation. Function may be isoform-specific: isoforms lacking the KTS motif may act as transcription factors. Isoforms containing the KTS motif may bind mRNA and play a role in mRNA metabolism or splicing. Isoform 1 has lower affinity for DNA, and can bind RNA.<ref>PMID:19123921</ref> <ref>PMID:19416806</ref> [[http://www.uniprot.org/uniprot/B2MG_HUMAN B2MG_HUMAN]] Component of the class I major histocompatibility complex (MHC). Involved in the presentation of peptide antigens to the immune system.
		+	== Evolutionary Conservation ==
		+	[[Image:Consurf_key_small.gif\|200px\|right]]
		+	Check<jmol>
		+	<jmolCheckbox>
		+	<scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/my/3myj_consurf.spt"</scriptWhenChecked>
		+	<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
		+	<text>to colour the structure by Evolutionary Conservation</text>
		+	</jmolCheckbox>
		+	</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary\|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/chain_selection.php?pdb_ID=2ata ConSurf].
		+	<div style="clear:both"></div>
		+	<div style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	Presentation of peptides by class I or class II major histocompatibility complex (MHC) molecules is required for the initiation and propagation of a T cell-mediated immune response. Peptides from the Wilms Tumor 1 transcription factor (WT1), upregulated in many hematopoetic and solid tumors, can be recognized by T cells and numerous efforts are underway to engineer WT1-based cancer vaccines. Here we determined the structures of the class I MHC molecule HLA-A*0201 bound to the native 126-134 epitope of the WT1 peptide and a recently described variant (R1Y) with improved MHC binding. The R1Y variant, a potential vaccine candidate, alters the positions of MHC charged side chains near the peptide N-terminus and significantly reduces the peptide/MHC electrostatic surface potential. These alterations indicate that the R1Y variant is an imperfect mimic of the native WT1 peptide, and suggest caution in its use as a therapeutic vaccine. Stability measurements revealed how the R1Y substitution enhances MHC binding affinity, and together with the structures suggest a strategy for engineering WT1 variants with improved MHC binding that retain the structural features of the native peptide/MHC complex.

-	~~==Disease==~~	+	Structures of native and affinity-enhanced WT1 epitopes bound to HLA-A*0201: implications for WT1-based cancer therapeutics.,Borbulevych OY, Do P, Baker BM Mol Immunol. 2010 Sep;47(15):2519-24. Epub 2010 Jul 8. PMID:20619457<ref>PMID:20619457</ref>
-	~~[[http://www.uniprot.org/uniprot/WT1_HUMAN WT1_HUMAN]] Defects in WT1 are the cause~~ of Frasier syndrome (FS) [MIM:[http://omim.org/entry/136680 136680]]. FS is characterized by a slowly progressing nephropathy leading to renal failure in adolescence or early adulthood, male pseudohermaphroditism, and ~~no Wilms tumor. As for histological findings of the kidneys, focal glomerular sclerosis is often observed. There is phenotypic overlap with Denys~~-~~Drash syndrome. Inheritance is autosomal dominant.<ref>PMID:10571943</ref> Defects in~~ WT1 are the cause of Wilms tumor 1 (WT1) [MIM:[http://omim.org/entry/194070 194070]]. WT is an embryonal malignancy of the kidney that affects approximately 1 in 10'000 infants and young children. It occurs both in sporadic and hereditary forms.<ref>PMID:1317572</ref><ref>PMID:9108089</ref><ref>PMID:9529364</ref><ref>PMID:15150775</ref> Defects in WT1 are the cause of Denys-~~Drash syndrome (DDS) [MIM~~:[http://omim.org/entry/194080 194080]]. DDS is a typical nephropathy characterized by diffuse mesangial sclerosis, genital abnormalities, and/or Wilms tumor. There is phenotypic overlap with WAGR syndrome and Frasier syndrome. Inheritance is autosomal dominant, but most cases are sporadic.<ref>PMID:9529364</ref><ref>PMID:1655284</ref><ref>PMID:1302008</ref><ref>PMID:1338906</ref><ref>PMID:8388765</ref><ref>PMID:8111391</ref><ref>PMID:8295405</ref><ref>PMID:8411073</ref><ref>PMID:8112732</ref><ref>PMID:8741319</ref><ref>PMID:8956030</ref><ref>PMID:9475094</ref><ref>PMID:10738002</ref><ref>PMID:11182928</ref><ref>PMID:10799199</ref><ref>PMID:11519891</ref><ref>PMID:15349765</ref> Defects in WT1 are the cause of nephrotic syndrome type 4 (NPHS4) [MIM:[http://omim.org/entry/256370 256370]]. A renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-~~stage renal failure~~. ~~Most patients with NPHS4 show diffuse mesangial sclerosis on renal biopsy~~, ~~which is a pathologic entity characterized by mesangial matrix expansion with no mesangial hypercellularity~~, ~~hypertrophy of the podocytes~~, ~~vacuolized podocytes, thickened basement membranes, and diminished patency of the capillary lumen~~.<ref>PMID:9529364</ref><ref>PMID:11182928</ref><ref>PMID:9607189</ref><ref>PMID:15253707</ref> Defects in WT1 are a cause of Meacham syndrome (MEACHS) [MIM:[http://omim.org/entry/608978 608978]]. Meacham syndrome is a rare sporadically occurring multiple malformation syndrome characterized by male pseudohermaphroditism with abnormal internal female genitalia comprising a uterus and double or septate vagina, complex congenital heart defect and diaphragmatic abnormalities.<ref>PMID:17853480</ref> Note=A chromosomal aberration involving WT1 may be a cause of desmoplastic small round cell tumor (DSRCT). Translocation t(11;~~22)~~(~~p13;q12~~) ~~with EWSR1. Defects in WT1 may be a cause of mesothelioma malignant (MESOM) [MIM~~:~~[http://omim.org/entry/156240 156240]]. An aggressive neoplasm of the serosal lining of the chest. It appears as broad sheets of cells, with some regions containing spindle~~-~~shaped, sarcoma-like cells and other regions showing adenomatous patterns~~. ~~Pleural mesotheliomas have been linked to exposure to asbestos~~.~~<ref>~~PMID:8401592</ref> [[http://www.uniprot.org/uniprot/B2MG_HUMAN B2MG_HUMAN]] Defects in B2M are the cause of hypercatabolic hypoproteinemia (HYCATHYP) [MIM:[http://omim.org/entry/241600 241600]]. Affected individuals show marked reduction in serum concentrations of immunoglobulin and albumin, probably due to rapid degradation.<ref>PMID:16549777</ref> Note=Beta-2-microglobulin may adopt the fibrillar configuration of amyloid in certain pathologic states. The capacity to assemble into amyloid fibrils is concentration dependent. Persistently high beta(2)-microglobulin serum levels lead to amyloidosis in patients on long-term hemodialysis.<ref>PMID:3532124</ref><ref>PMID:1336137</ref><ref>PMID:7554280</ref><ref>PMID:4586824</ref><ref>PMID:8084451</ref><ref>PMID:12119416</ref><ref>PMID:12796775</ref><ref>PMID:16901902</ref><ref>PMID:16491088</ref><ref>PMID:17646174</ref><ref>PMID:18835253</ref><ref>PMID:18395224</ref><ref>PMID:~~19284997~~</ref>	+

-	~~==Function==~~	+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
-	~~[[http:~~/~~/www.uniprot.org/uniprot/1A02_HUMAN 1A02_HUMAN]] Involved in the presentation~~ of ~~foreign antigens to~~ the ~~immune system~~. ~~[[http://www~~.~~uniprot.org/uniprot/WT1_HUMAN WT1_HUMAN]] Transcription factor that plays an important role in cellular development and cell survival. Regulates the expression~~ of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. Recognizes and binds to the DNA sequence 5'-CGCCCCCGC-3'. It has a tumor suppressor as well as an oncogenic role in tumor formation. Function may be isoform-specific: isoforms lacking the KTS motif may act as transcription factors. Isoforms containing the KTS motif may bind mRNA and play a role in mRNA metabolism or splicing. Isoform 1 has lower affinity for DNA, and can bind RNA.<~~ref~~>~~PMID:19123921~~</~~ref~~><ref>PMID:19416806</ref> [[http://www.uniprot.org/uniprot/B2MG_HUMAN B2MG_HUMAN]] Component of the class I major histocompatibility complex (MHC). Involved in the presentation of peptide antigens to the immune system.	+	</div>
-		+
-	~~==About this Structure==~~	+
-	[[3myj]] is a 6 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=3MYJ OCA].	+

	==See Also==		==See Also==
	*[[Beta-2 microglobulin\|Beta-2 microglobulin]]		*[[Beta-2 microglobulin\|Beta-2 microglobulin]]
	*[[Major histocompatibility complex\|Major histocompatibility complex]]		*[[Major histocompatibility complex\|Major histocompatibility complex]]
-		+	== References ==
-	==~~Reference~~==	+	<references/>
-	~~<ref group="xtra">PMID:020619457</ref>~~<references ~~group="xtra"~~/><~~references~~/>	+	__TOC__
		+	</StructureSection>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
-	[[Category: Baker, B M.]]	+	[[Category: Baker, B M]]
-	[[Category: Borbulevych, O Y.]]	+	[[Category: Borbulevych, O Y]]
	[[Category: Cancer vaccine]]		[[Category: Cancer vaccine]]
	[[Category: Disease mutation]]		[[Category: Disease mutation]]

OCA at 05:54, 25 March 2013

OCA — Mon, 25 Mar 2013 05:54:07 GMT

←Older revision		Revision as of 05:54, 25 March 2013
Line 1:		Line 1:
-	[[Image:3myj.png\|left\|200px]]
-
	{{STRUCTURE_3myj\| PDB=3myj \| SCENE= }}		{{STRUCTURE_3myj\| PDB=3myj \| SCENE= }}
-
	===Human Class I MHC HLA-A2 in complex with the WT-1 (126-134) (R1Y) peptide variant.===		===Human Class I MHC HLA-A2 in complex with the WT-1 (126-134) (R1Y) peptide variant.===
		+	{{ABSTRACT_PUBMED_20619457}}

-	~~{{ABSTRACT_PUBMED_20619457}}~~	+	==Disease==
		+	[[http://www.uniprot.org/uniprot/WT1_HUMAN WT1_HUMAN]] Defects in WT1 are the cause of Frasier syndrome (FS) [MIM:[http://omim.org/entry/136680 136680]]. FS is characterized by a slowly progressing nephropathy leading to renal failure in adolescence or early adulthood, male pseudohermaphroditism, and no Wilms tumor. As for histological findings of the kidneys, focal glomerular sclerosis is often observed. There is phenotypic overlap with Denys-Drash syndrome. Inheritance is autosomal dominant.<ref>PMID:10571943</ref> Defects in WT1 are the cause of Wilms tumor 1 (WT1) [MIM:[http://omim.org/entry/194070 194070]]. WT is an embryonal malignancy of the kidney that affects approximately 1 in 10'000 infants and young children. It occurs both in sporadic and hereditary forms.<ref>PMID:1317572</ref><ref>PMID:9108089</ref><ref>PMID:9529364</ref><ref>PMID:15150775</ref> Defects in WT1 are the cause of Denys-Drash syndrome (DDS) [MIM:[http://omim.org/entry/194080 194080]]. DDS is a typical nephropathy characterized by diffuse mesangial sclerosis, genital abnormalities, and/or Wilms tumor. There is phenotypic overlap with WAGR syndrome and Frasier syndrome. Inheritance is autosomal dominant, but most cases are sporadic.<ref>PMID:9529364</ref><ref>PMID:1655284</ref><ref>PMID:1302008</ref><ref>PMID:1338906</ref><ref>PMID:8388765</ref><ref>PMID:8111391</ref><ref>PMID:8295405</ref><ref>PMID:8411073</ref><ref>PMID:8112732</ref><ref>PMID:8741319</ref><ref>PMID:8956030</ref><ref>PMID:9475094</ref><ref>PMID:10738002</ref><ref>PMID:11182928</ref><ref>PMID:10799199</ref><ref>PMID:11519891</ref><ref>PMID:15349765</ref> Defects in WT1 are the cause of nephrotic syndrome type 4 (NPHS4) [MIM:[http://omim.org/entry/256370 256370]]. A renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. Most patients with NPHS4 show diffuse mesangial sclerosis on renal biopsy, which is a pathologic entity characterized by mesangial matrix expansion with no mesangial hypercellularity, hypertrophy of the podocytes, vacuolized podocytes, thickened basement membranes, and diminished patency of the capillary lumen.<ref>PMID:9529364</ref><ref>PMID:11182928</ref><ref>PMID:9607189</ref><ref>PMID:15253707</ref> Defects in WT1 are a cause of Meacham syndrome (MEACHS) [MIM:[http://omim.org/entry/608978 608978]]. Meacham syndrome is a rare sporadically occurring multiple malformation syndrome characterized by male pseudohermaphroditism with abnormal internal female genitalia comprising a uterus and double or septate vagina, complex congenital heart defect and diaphragmatic abnormalities.<ref>PMID:17853480</ref> Note=A chromosomal aberration involving WT1 may be a cause of desmoplastic small round cell tumor (DSRCT). Translocation t(11;22)(p13;q12) with EWSR1. Defects in WT1 may be a cause of mesothelioma malignant (MESOM) [MIM:[http://omim.org/entry/156240 156240]]. An aggressive neoplasm of the serosal lining of the chest. It appears as broad sheets of cells, with some regions containing spindle-shaped, sarcoma-like cells and other regions showing adenomatous patterns. Pleural mesotheliomas have been linked to exposure to asbestos.<ref>PMID:8401592</ref> [[http://www.uniprot.org/uniprot/B2MG_HUMAN B2MG_HUMAN]] Defects in B2M are the cause of hypercatabolic hypoproteinemia (HYCATHYP) [MIM:[http://omim.org/entry/241600 241600]]. Affected individuals show marked reduction in serum concentrations of immunoglobulin and albumin, probably due to rapid degradation.<ref>PMID:16549777</ref> Note=Beta-2-microglobulin may adopt the fibrillar configuration of amyloid in certain pathologic states. The capacity to assemble into amyloid fibrils is concentration dependent. Persistently high beta(2)-microglobulin serum levels lead to amyloidosis in patients on long-term hemodialysis.<ref>PMID:3532124</ref><ref>PMID:1336137</ref><ref>PMID:7554280</ref><ref>PMID:4586824</ref><ref>PMID:8084451</ref><ref>PMID:12119416</ref><ref>PMID:12796775</ref><ref>PMID:16901902</ref><ref>PMID:16491088</ref><ref>PMID:17646174</ref><ref>PMID:18835253</ref><ref>PMID:18395224</ref><ref>PMID:19284997</ref>
		+
		+	==Function==
		+	[[http://www.uniprot.org/uniprot/1A02_HUMAN 1A02_HUMAN]] Involved in the presentation of foreign antigens to the immune system. [[http://www.uniprot.org/uniprot/WT1_HUMAN WT1_HUMAN]] Transcription factor that plays an important role in cellular development and cell survival. Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. Recognizes and binds to the DNA sequence 5'-CGCCCCCGC-3'. It has a tumor suppressor as well as an oncogenic role in tumor formation. Function may be isoform-specific: isoforms lacking the KTS motif may act as transcription factors. Isoforms containing the KTS motif may bind mRNA and play a role in mRNA metabolism or splicing. Isoform 1 has lower affinity for DNA, and can bind RNA.<ref>PMID:19123921</ref><ref>PMID:19416806</ref> [[http://www.uniprot.org/uniprot/B2MG_HUMAN B2MG_HUMAN]] Component of the class I major histocompatibility complex (MHC). Involved in the presentation of peptide antigens to the immune system.

	==About this Structure==		==About this Structure==
Line 15:		Line 17:

	==Reference==		==Reference==
-	<ref group="xtra">PMID:020619457</ref><references group="xtra"/>	+	<ref group="xtra">PMID:020619457</ref><references group="xtra"/><references/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Baker, B M.]]		[[Category: Baker, B M.]]

OCA at 09:12, 20 October 2012

OCA — Sat, 20 Oct 2012 09:12:29 GMT

←Older revision		Revision as of 09:12, 20 October 2012
Line 8:		Line 8:

	==About this Structure==		==About this Structure==
-	[[3myj]] is a 6 chain structure ~~of [[Beta-2 microglobulin]] and [[Major histocompatibility complex]]~~ 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=3MYJ OCA].	+	[[3myj]] is a 6 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=3MYJ OCA].

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

OCA: Protected "3myj" [edit=sysop:move=sysop]

OCA — Wed, 25 Jul 2012 22:06:43 GMT

Protected "3myj" [edit=sysop:move=sysop]

←Older revision

Revision as of 22:06, 25 July 2012

OCA at 22:06, 25 July 2012

OCA — Wed, 25 Jul 2012 22:06:40 GMT

←Older revision		Revision as of 22:06, 25 July 2012
Line 1:		Line 1:
-	~~{{Seed}}~~	+	[[Image:3myj.png\|left\|200px]]
-	[[Image:3myj.~~jpg~~\|left\|200px]]	+

-	<!--
-	The line below this paragraph, containing "STRUCTURE_3myj", 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_3myj\| PDB=3myj \| SCENE= }}		{{STRUCTURE_3myj\| PDB=3myj \| SCENE= }}

	===Human Class I MHC HLA-A2 in complex with the WT-1 (126-134) (R1Y) peptide variant.===		===Human Class I MHC HLA-A2 in complex with the WT-1 (126-134) (R1Y) peptide variant.===

-
-	<!--
-	The line below this paragraph, {{ABSTRACT_PUBMED_20619457}}, adds the Publication Abstract to the page
-	(as it appears on PubMed at http://www.pubmed.gov), where 20619457 is the PubMed ID number.
-	-->
	{{ABSTRACT_PUBMED_20619457}}		{{ABSTRACT_PUBMED_20619457}}

	==About this Structure==		==About this Structure==
-	~~3MYJ~~ is a 6 ~~chains~~ structure with ~~sequences~~ 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=3MYJ OCA].	+	[[3myj]] is a 6 chain structure of [[Beta-2 microglobulin]] and [[Major histocompatibility complex]] 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=3MYJ OCA].
		+
		+	==See Also==
		+	*[[Beta-2 microglobulin\|Beta-2 microglobulin]]
		+	*[[Major histocompatibility complex\|Major histocompatibility complex]]

	==Reference==		==Reference==
-	<ref group="xtra">PMID:~~20619457~~</ref><references group="xtra"/>	+	<ref group="xtra">PMID:020619457</ref><references group="xtra"/>
	[[Category: Homo sapiens]]		[[Category: Homo sapiens]]
	[[Category: Baker, B M.]]		[[Category: Baker, B M.]]
Line 47:		Line 39:
	[[Category: Transmembrane]]		[[Category: Transmembrane]]
	[[Category: Wt-1 peptide]]		[[Category: Wt-1 peptide]]
-
-	''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Aug 18 11:46:21 2010''

OCA at 07:38, 18 August 2010

OCA — Wed, 18 Aug 2010 07:38:50 GMT

←Older revision		Revision as of 07:38, 18 August 2010
Line 1:		Line 1:
-	~~'''Unreleased structure'''~~	+	{{Seed}}
		+	[[Image:3myj.jpg\|left\|200px]]

-	The ~~entry 3myj~~ is ~~ON HOLD~~	+	<!--
		+	The line below this paragraph, containing "STRUCTURE_3myj", 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_3myj\| PDB=3myj \| SCENE= }}

-	~~Authors: Borbulevych, O.Y., Baker, B.M~~.	+	===Human Class I MHC HLA-A2 in complex with the WT-1 (126-134) (R1Y) peptide variant.===

-	Description: Human Class I MHC HLA-A2 in complex with the WT-1 (126-134) (R1Y) peptide variant.

-	''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on ~~Thu May 20 09~~:19:36 2010''	+	<!--
		+	The line below this paragraph, {{ABSTRACT_PUBMED_20619457}}, adds the Publication Abstract to the page
		+	(as it appears on PubMed at http://www.pubmed.gov), where 20619457 is the PubMed ID number.
		+	-->
		+	{{ABSTRACT_PUBMED_20619457}}
		+
		+	==About this Structure==
		+	3MYJ is a 6 chains structure with sequences 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=3MYJ OCA].
		+
		+	==Reference==
		+	<ref group="xtra">PMID:20619457</ref><references group="xtra"/>
		+	[[Category: Homo sapiens]]
		+	[[Category: Baker, B M.]]
		+	[[Category: Borbulevych, O Y.]]
		+	[[Category: Cancer vaccine]]
		+	[[Category: Disease mutation]]
		+	[[Category: Disulfide bond]]
		+	[[Category: Glycation]]
		+	[[Category: Glycoprotein]]
		+	[[Category: Hla-a2]]
		+	[[Category: Host-virus interaction]]
		+	[[Category: Immune response]]
		+	[[Category: Immune system]]
		+	[[Category: Immunoglobulin domain]]
		+	[[Category: Membrane]]
		+	[[Category: Mhc class i]]
		+	[[Category: Mhc i]]
		+	[[Category: Nonapeptide]]
		+	[[Category: Phosphoprotein]]
		+	[[Category: Pyrrolidone carboxylic acid]]
		+	[[Category: R1y mutation]]
		+	[[Category: Secreted]]
		+	[[Category: Transmembrane]]
		+	[[Category: Wt-1 peptide]]
		+
		+	''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Aug 18 11:46:21 2010''