6gk3

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'''Unreleased structure'''
 
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The entry 6gk3 is ON HOLD until Paper Publication
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==Two protofilament beta-2-microglobulin amyloid fibril==
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<StructureSection load='6gk3' size='340' side='right' caption='[[6gk3]], [[Resolution|resolution]] 3.90&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[6gk3]] is a 8 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6GK3 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6GK3 FirstGlance]. <br>
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</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=6gk3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6gk3 OCA], [http://pdbe.org/6gk3 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6gk3 RCSB], [http://www.ebi.ac.uk/pdbsum/6gk3 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6gk3 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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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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== Function ==
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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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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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All amyloid fibrils contain a cross-beta fold. How this structure differs in fibrils formed from proteins associated with different diseases remains unclear. Here, we combine cryo-EM and MAS-NMR to determine the structure of an amyloid fibril formed in vitro from beta2-microglobulin (beta2m), the culprit protein of dialysis-related amyloidosis. The fibril is composed of two identical protofilaments assembled from subunits that do not share beta2m's native tertiary fold, but are formed from similar beta-strands. The fibrils share motifs with other amyloid fibrils, but also contain unique features including pi-stacking interactions perpendicular to the fibril axis and an intramolecular disulfide that stabilises the subunit fold. We also describe a structural model for a second fibril morphology and show that it is built from the same subunit fold. The results provide insights into the mechanisms of fibril formation and the commonalities and differences within the amyloid fold in different protein sequences.
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Authors:
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The structure of a beta2-microglobulin fibril suggests a molecular basis for its amyloid polymorphism.,Iadanza MG, Silvers R, Boardman J, Smith HI, Karamanos TK, Debelouchina GT, Su Y, Griffin RG, Ranson NA, Radford SE Nat Commun. 2018 Oct 30;9(1):4517. doi: 10.1038/s41467-018-06761-6. PMID:30375379<ref>PMID:30375379</ref>
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Description:
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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[[Category: Unreleased Structures]]
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</div>
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<div class="pdbe-citations 6gk3" style="background-color:#fffaf0;"></div>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
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[[Category: Iadanza, M G]]
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[[Category: Ranson, N A]]
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[[Category: Amyloid]]
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[[Category: B2m]]
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[[Category: Protein fibril]]

Revision as of 08:19, 14 November 2018

Two protofilament beta-2-microglobulin amyloid fibril

6gk3, resolution 3.90Å

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