6m1b: Difference between revisions
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<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'> | <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 [https://proteopedia.org/fgij/fg.htm?mol=6M1B FirstGlance]. <br> | ||
</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> | </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='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='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> | ||
<div style="background-color:#fffaf0;"> | <div style="background-color:#fffaf0;"> | ||
== Publication Abstract from PubMed == | == Publication Abstract from PubMed == | ||
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__TOC__ | __TOC__ | ||
</StructureSection> | </StructureSection> | ||
[[Category: Large Structures]] | [[Category: Large Structures]] | ||
[[Category: Goto Y]] | [[Category: Goto Y]] | ||
Latest revision as of 11:07, 17 October 2024
A new V27M variant of beta 2 microglobulin induced amyloidosis in a patient with long-term hemodialysisA new V27M variant of beta 2 microglobulin induced amyloidosis in a patient with long-term hemodialysis
Structural highlights
Publication Abstract from PubMedTill 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[1] From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine. See AlsoReferences
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