6m1b - Revision history

OCA at 15:10, 29 November 2023

2023-11-29T15:10:09Z

←Older revision		Revision as of 15:10, 29 November 2023
Line 3:		Line 3:
	<StructureSection load='6m1b' size='340' side='right'caption='[[6m1b]], [[Resolution\|resolution]] 1.88Å' scene=''>		<StructureSection load='6m1b' size='340' side='right'caption='[[6m1b]], [[Resolution\|resolution]] 1.88Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[6m1b]] is a 1 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=6M1B OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6M1B FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[6m1b]] 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=6M1B OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6M1B 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=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=PO4:PHOSPHATE+ION'>PO4</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]] 1.88Å</td></tr>
-	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">B2M, CDABP0092, HDCMA22P ([https://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</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=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=PO4:PHOSPHATE+ION'>PO4</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=6m1b FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6m1b OCA], [https://pdbe.org/6m1b PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6m1b RCSB], [https://www.ebi.ac.uk/pdbsum/6m1b PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6m1b 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=6m1b FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6m1b OCA], [https://pdbe.org/6m1b PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6m1b RCSB], [https://www.ebi.ac.uk/pdbsum/6m1b PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6m1b ProSAT]</span></td></tr>
	</table>		</table>
	== Disease ==		== Disease ==
-	[[https://www.uniprot.org/uniprot/B2MG_HUMAN B2MG_HUMAN]] Defects in B2M are the cause of hypercatabolic hypoproteinemia (HYCATHYP) [MIM:[https://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>	+	[https://www.uniprot.org/uniprot/B2MG_HUMAN B2MG_HUMAN] Defects in B2M are the cause of hypercatabolic hypoproteinemia (HYCATHYP) [MIM:[https://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 ==		== Function ==
-	[[https://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.	+	[https://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 style="background-color:#fffaf0;">		<div style="background-color:#fffaf0;">
	== Publication Abstract from PubMed ==		== Publication Abstract from PubMed ==
Line 21:		Line 21:
	</div>		</div>
	<div class="pdbe-citations 6m1b" style="background-color:#fffaf0;"></div>		<div class="pdbe-citations 6m1b" style="background-color:#fffaf0;"></div>
		+
		+	==See Also==
		+	*[[Beta-2 microglobulin 3D structures\|Beta-2 microglobulin 3D structures]]
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
-	[[Category: ~~Human~~]]	+	[[Category: Homo sapiens]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	[[Category: Goto, Y]]	+	[[Category: Goto Y]]
-	[[Category: Kurisu, G]]	+	[[Category: Kurisu G]]
-	[[Category: Nakahara, S]]	+	[[Category: Nakahara S]]
-	[[Category: Nakaniwa, T]]	+	[[Category: Nakaniwa T]]
-	[[Category: So, M]]	+	[[Category: So M]]
-	[[Category: Tanaka, H]]	+	[[Category: Tanaka H]]
-	~~[[Category: Amyloidosis]]~~	+
-	~~[[Category: Beta 2 microglobulin]]~~	+
-	~~[[Category: Hemodialysis]]~~	+
-	~~[[Category: Immune system~~]]	+

OCA at 06:48, 24 March 2021

2021-03-24T06:48:00Z

←Older revision		Revision as of 06:48, 24 March 2021
Line 1:		Line 1:

	==A new V27M variant of beta 2 microglobulin induced amyloidosis in a patient with long-term hemodialysis==		==A new V27M variant of beta 2 microglobulin induced amyloidosis in a patient with long-term hemodialysis==
-	<StructureSection load='6m1b' size='340' side='right'caption='[[6m1b]]' scene=''>	+	<StructureSection load='6m1b' size='340' side='right'caption='[[6m1b]], [[Resolution\|resolution]] 1.88Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6M1B OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://proteopedia.org/fgij/fg.htm?mol=6M1B FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[6m1b]] is a 1 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=6M1B OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6M1B FirstGlance]. <br>
-	</td></tr><tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[~~http~~://proteopedia.org/fgij/fg.htm?mol=6m1b FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6m1b OCA], [~~http~~://pdbe.org/6m1b PDBe], [~~http~~://www.rcsb.org/pdb/explore.do?structureId=6m1b RCSB], [~~http~~://www.ebi.ac.uk/pdbsum/6m1b PDBsum], [~~http~~://prosat.h-its.org/prosat/prosatexe?pdbcode=6m1b ProSAT]</span></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=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=PO4:PHOSPHATE+ION'>PO4</scene></td></tr>
		+	<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">B2M, CDABP0092, HDCMA22P ([https://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
		+	<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=6m1b FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6m1b OCA], [https://pdbe.org/6m1b PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6m1b RCSB], [https://www.ebi.ac.uk/pdbsum/6m1b PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6m1b ProSAT]</span></td></tr>
	</table>		</table>
		+	== Disease ==
		+	[[https://www.uniprot.org/uniprot/B2MG_HUMAN B2MG_HUMAN]] Defects in B2M are the cause of hypercatabolic hypoproteinemia (HYCATHYP) [MIM:[https://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 ==
		+	[[https://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 style="background-color:#fffaf0;">
		+	== Publication Abstract from PubMed ==
		+	Till date, there had been no reported case of dialysis-related amyloidosis (DRA) associated with a beta2-microglobulin variant. We report here a 41-year-old haemodialysis patient with systemic amyloidosis, exhibiting macroglossia and swelling salivary glands, uncommon clinical manifestations for DRA. Molecular analysis showed that the patient had a new variant of beta2-microglobulin (V27M). Extracted amyloid protein was predominantly composed of variant beta2-microglobulin. In vitro analysis revealed that this variant beta2-microglobulin had a strong amyloidogenic propensity, probably owing to the decreased stability caused by a bulky methionine residue. Our data clearly show that V27M variant is amyloidogenic and this mutation results in unusual clinical manifestations. To date, only one amyloidogenic beta2-microglobulin variant (D76N) has been reported in non-dialysis patients. It is noteworthy that the V27M and D76N variants show substantial differences in both clinical phenotypes and pathomechanical features. This is the first case of DRA associated with a naturally occurring beta2-microglobulin variant.
		+
		+	Dialysis-related amyloidosis associated with a novel beta2-microglobulin variant.,Mizuno H, Hoshino J, So M, Kogure Y, Fujii T, Ubara Y, Takaichi K, Nakaniwa T, Tanaka H, Kurisu G, Kametani F, Nakagawa M, Yoshinaga T, Sekijima Y, Higuchi K, Goto Y, Yazaki M Amyloid. 2020 Sep 2:1-8. doi: 10.1080/13506129.2020.1813097. PMID:32875920<ref>PMID:32875920</ref>
		+
		+	From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
		+	</div>
		+	<div class="pdbe-citations 6m1b" style="background-color:#fffaf0;"></div>
		+	== References ==
		+	<references/>
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
		+	[[Category: Human]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	[[Category: Goto Y]]	+	[[Category: Goto, Y]]
-	[[Category: Kurisu G]]	+	[[Category: Kurisu, G]]
-	[[Category: Nakahara S]]	+	[[Category: Nakahara, S]]
-	[[Category: Nakaniwa T]]	+	[[Category: Nakaniwa, T]]
-	[[Category: So M]]	+	[[Category: So, M]]
-	[[Category: Tanaka H]]	+	[[Category: Tanaka, H]]
		+	[[Category: Amyloidosis]]
		+	[[Category: Beta 2 microglobulin]]
		+	[[Category: Hemodialysis]]
		+	[[Category: Immune system]]

OCA at 08:52, 6 January 2021

2021-01-06T08:52:38Z

←Older revision		Revision as of 08:52, 6 January 2021
Line 1:		Line 1:
-	'''Unreleased structure'''

-	~~The entry 6m1b is ON HOLD~~	+	==A new V27M variant of beta 2 microglobulin induced amyloidosis in a patient with long-term hemodialysis==
-		+	<StructureSection load='6m1b' size='340' side='right'caption='[[6m1b]]' scene=''>
-	~~Authors: So, M., Nakahara, S., Nakaniwa, T., Tanaka, H., Kurisu, G., Goto, Y.~~	+	== Structural highlights ==
-		+	<table><tr><td colspan='2'>Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6M1B OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/fgij/fg.htm?mol=6M1B FirstGlance]. <br>
-	~~Description:~~ A new V27M variant of beta 2 microglobulin induced amyloidosis in a patient with long-term hemodialysis	+	</td></tr><tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/fgij/fg.htm?mol=6m1b FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6m1b OCA], [http://pdbe.org/6m1b PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6m1b RCSB], [http://www.ebi.ac.uk/pdbsum/6m1b PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6m1b ProSAT]</span></td></tr>
-	[[Category: ~~Unreleased~~ Structures]]	+	</table>
-	[[Category: ~~Tanaka, H~~]]	+	__TOC__
-	[[Category: Kurisu, G]]	+	</StructureSection>
-	[[Category: ~~Goto, Y~~]]	+	[[Category: Large Structures]]
-	[[Category: Nakaniwa, T]]	+	[[Category: Goto Y]]
-	[[Category: So, M]]	+	[[Category: Kurisu G]]
-	[[Category: ~~Nakahara, S~~]]	+	[[Category: Nakahara S]]
		+	[[Category: Nakaniwa T]]
		+	[[Category: So M]]
		+	[[Category: Tanaka H]]

OCA: Protected "6m1b" [edit=sysop:move=sysop]

2020-03-04T05:29:28Z

Protected "6m1b" [edit=sysop:move=sysop]

←Older revision

Revision as of 05:29, 4 March 2020

OCA: New page: '''Unreleased structure''' The entry 6m1b is ON HOLD Authors: So, M., Nakahara, S., Nakaniwa, T., Tanaka, H., Kurisu, G., Goto, Y. Description: A new V27M variant of beta 2 microglobul...

2020-03-04T05:29:27Z

New page: '''Unreleased structure''' The entry 6m1b is ON HOLD Authors: So, M., Nakahara, S., Nakaniwa, T., Tanaka, H., Kurisu, G., Goto, Y. Description: A new V27M variant of beta 2 microglobul...

New page

'''Unreleased structure'''

The entry 6m1b is ON HOLD

Authors: So, M., Nakahara, S., Nakaniwa, T., Tanaka, H., Kurisu, G., Goto, Y.

Description: A new V27M variant of beta 2 microglobulin induced amyloidosis in a patient with long-term hemodialysis
[[Category: Unreleased Structures]]
[[Category: Tanaka, H]]
[[Category: Kurisu, G]]
[[Category: Goto, Y]]
[[Category: Nakaniwa, T]]
[[Category: So, M]]
[[Category: Nakahara, S]]