4kdt

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{{STRUCTURE_4kdt| PDB=4kdt | SCENE= }}
 
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===Structure of an early native-like intermediate of beta2-microglobulin amyloidosis===
 
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{{ABSTRACT_PUBMED_23904325}}
 
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==Disease==
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==Structure of an early native-like intermediate of beta2-microglobulin amyloidosis==
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[[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>
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<StructureSection load='4kdt' size='340' side='right'caption='[[4kdt]], [[Resolution|resolution]] 2.60&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[4kdt]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Camelidae Camelidae] and [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4KDT OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4KDT FirstGlance]. <br>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.6&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=4kdt FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4kdt OCA], [https://pdbe.org/4kdt PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4kdt RCSB], [https://www.ebi.ac.uk/pdbsum/4kdt PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4kdt ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[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>
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== Function ==
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[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.
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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To investigate early intermediates of beta2-microglobulin (beta2m) amyloidogenesis, we solved the structure of beta2m containing the amyloidogenic Pro32Gly mutation by X-ray crystallography. One nanobody (Nb24) that efficiently blocks fibril elongation was used as a chaperone to co-crystallize the Pro32Gly beta2m monomer under physiological conditions. The complex of P32G beta2m with Nb24 reveals a trans peptide bond at position 32 of this amyloidogenic variant, whereas Pro32 adopts the cis conformation in the wild-type monomer, indicating that the cis to trans isomerization at Pro32 plays a critical role in the early onset of beta2m amyloid formation.
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==Function==
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Structure of an early native-like intermediate of beta2-microglobulin amyloidogenesis.,Vanderhaegen S, Fislage M, Domanska K, Versees W, Pardon E, Bellotti V, Steyaert J Protein Sci. 2013 Jul 31. doi: 10.1002/pro.2321. PMID:23904325<ref>PMID:23904325</ref>
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[[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.
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==About this Structure==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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[[4kdt]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Camelidae Camelidae] and [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4KDT OCA].
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</div>
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<div class="pdbe-citations 4kdt" style="background-color:#fffaf0;"></div>
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==Reference==
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==See Also==
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<ref group="xtra">PMID:023904325</ref><references group="xtra"/><references/>
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*[[Beta-2 microglobulin 3D structures|Beta-2 microglobulin 3D structures]]
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Camelidae]]
[[Category: Camelidae]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Fislage, M.]]
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[[Category: Large Structures]]
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[[Category: Pardon, E.]]
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[[Category: Fislage M]]
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[[Category: Steyaert, J.]]
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[[Category: Pardon E]]
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[[Category: Vanderhaegen, S.]]
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[[Category: Steyaert J]]
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[[Category: Versees, W.]]
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[[Category: Vanderhaegen S]]
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[[Category: Immune system]]
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[[Category: Versees W]]
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[[Category: Immunoglobulin fold]]
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Current revision

Structure of an early native-like intermediate of beta2-microglobulin amyloidosis

PDB ID 4kdt

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