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2x89

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{{STRUCTURE_2x89| PDB=2x89 | SCENE= }}
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==Structure of the Beta2_microglobulin involved in amyloidogenesis==
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===Structure of the Beta2_microglobulin involved in amyloidogenesis===
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<StructureSection load='2x89' size='340' side='right' caption='[[2x89]], [[Resolution|resolution]] 2.16&Aring;' scene=''>
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{{ABSTRACT_PUBMED_21220305}}
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== Structural highlights ==
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<table><tr><td colspan='2'>[[2x89]] is a 7 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=2X89 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2X89 FirstGlance]. <br>
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==Disease==
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</td></tr><tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1uqs|1uqs]], [[1bd2|1bd2]], [[2esv|2esv]], [[2xks|2xks]], [[2ak4|2ak4]], [[1ypz|1ypz]], [[1im3|1im3]], [[1i7u|1i7u]], [[1uxw|1uxw]], [[1c16|1c16]], [[1hsa|1hsa]], [[2axf|2axf]], [[1gzp|1gzp]], [[2bnq|2bnq]], [[1w72|1w72]], [[2jcc|2jcc]], [[2bck|2bck]], [[1de4|1de4]], [[2vlk|2vlk]], [[1exu|1exu]], [[1qrn|1qrn]], [[2hla|2hla]], [[1mhe|1mhe]], [[1eez|1eez]], [[1im9|1im9]], [[1jht|1jht]], [[1qqd|1qqd]], [[1qr1|1qr1]], [[1zs8|1zs8]], [[1hla|1hla]], [[1jgd|1jgd]], [[1i1y|1i1y]], [[1vgk|1vgk]], [[1age|1age]], [[1ur7|1ur7]], [[1s9x|1s9x]], [[1hhg|1hhg]], [[2x4u|2x4u]], [[1duz|1duz]], [[1a9e|1a9e]], [[2clr|2clr]], [[3hla|3hla]], [[1m05|1m05]], [[2x4o|2x4o]], [[1tvb|1tvb]], [[2v2w|2v2w]], [[1onq|1onq]], [[2x4p|2x4p]], [[2vlr|2vlr]], [[2bvo|2bvo]], [[1a1n|1a1n]], [[1lp9|1lp9]], [[1zsd|1zsd]], [[1m6o|1m6o]], [[1hhk|1hhk]], [[1zt4|1zt4]], [[1hsb|1hsb]], [[1ce6|1ce6]], [[2xku|2xku]], [[1x7q|1x7q]], [[1py4|1py4]], [[1syv|1syv]], [[2j8u|2j8u]], [[2xpg|2xpg]], [[1sys|1sys]], [[1ogt|1ogt]], [[2x4t|2x4t]], [[1cg9|1cg9]], [[1p7q|1p7q]], [[1q94|1q94]], [[1jnj|1jnj]], [[1agb|1agb]], [[2d31|2d31]], [[1xz0|1xz0]], [[1lds|1lds]], [[1hhh|1hhh]], [[1tvh|1tvh]], [[1xr8|1xr8]], [[2bss|2bss]], [[1a1m|1a1m]], [[1e28|1e28]], [[2bvp|2bvp]], [[2v2x|2v2x]], [[1xr9|1xr9]], [[2gj6|2gj6]], [[2x4r|2x4r]], [[1qlf|1qlf]], [[1efx|1efx]], [[1tmc|1tmc]], [[2av1|2av1]], [[1qsf|1qsf]], [[1kpr|1kpr]], [[1duy|1duy]], [[1jge|1jge]], [[2hjl|2hjl]], [[1qew|1qew]], [[1w0v|1w0v]], [[1k5n|1k5n]], [[1ao7|1ao7]], [[2bnr|2bnr]], [[1xh3|1xh3]], [[2bst|2bst]], [[1mi5|1mi5]], [[2h26|2h26]], [[1s9y|1s9y]], [[1a1o|1a1o]], [[2a83|2a83]], [[1agf|1agf]], [[1oga|1oga]], [[2f8o|2f8o]], [[2x70|2x70]], [[2cii|2cii]], [[1i7r|1i7r]], [[1jf1|1jf1]], [[2c7u|2c7u]], [[1e27|1e27]], [[2f74|2f74]], [[1w0w|1w0w]], [[1gzq|1gzq]], [[1uxs|1uxs]], [[1akj|1akj]], [[2hjk|2hjk]], [[2vb5|2vb5]], [[1agd|1agd]], [[2x4n|2x4n]], [[1r3h|1r3h]], [[1eey|1eey]], [[1i7t|1i7t]], [[1ydp|1ydp]], [[1i4f|1i4f]], [[2vll|2vll]], [[2bsr|2bsr]], [[2vlj|2vlj]], [[1b0g|1b0g]], [[2x4s|2x4s]], [[1b0r|1b0r]], [[1of2|1of2]], [[1hhi|1hhi]], [[1qse|1qse]], [[1a9b|1a9b]], [[2axg|2axg]], [[2bvq|2bvq]], [[1agc|1agc]], [[1qvo|1qvo]], [[1hhj|1hhj]], [[1s9w|1s9w]], [[1ktl|1ktl]], [[1a6z|1a6z]], [[2cik|2cik]], [[1i1f|1i1f]], [[2uwe|2uwe]], [[2av7|2av7]]</td></tr>
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<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=2x89 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2x89 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2x89 RCSB], [http://www.ebi.ac.uk/pdbsum/2x89 PDBsum]</span></td></tr>
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</table>
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== Disease ==
[[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>
[[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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==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.
[[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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Atomic-level structural investigation of the key conformational intermediates of amyloidogenesis remains a challenge. Here we demonstrate the utility of nanobodies to trap and characterize intermediates of beta2-microglobulin (beta2m) amyloidogenesis by X-ray crystallography. For this purpose, we selected five single domain antibodies that block the fibrillogenesis of a proteolytic amyloidogenic fragment of beta2m (DeltaN6beta2m). The crystal structure of DeltaN6beta2m in complex with one of these nanobodies (Nb24) identifies domain swapping as a plausible mechanism of self-association of this amyloidogenic protein. In the swapped dimer, two extended hinge loops-corresponding to the heptapetide NHVTLSQ that forms amyloid in isolation-are unmasked and fold into a new two-stranded antiparallel beta-sheet. The beta-strands of this sheet are prone to self-associate and stack perpendicular to the direction of the strands to build large intermolecular beta-sheets that run parallel to the axis of growing oligomers, providing an elongation mechanism by self-templated growth.
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==About this Structure==
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Atomic structure of a nanobody-trapped domain-swapped dimer of an amyloidogenic {beta}2-microglobulin variant.,Domanska K, Vanderhaegen S, Srinivasan V, Pardon E, Dupeux F, Marquez JA, Giorgetti S, Stoppini M, Wyns L, Bellotti V, Steyaert J Proc Natl Acad Sci U S A. 2011 Jan 10. PMID:21220305<ref>PMID:21220305</ref>
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[[2x89]] is a 7 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=2X89 OCA].
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
==See Also==
==See Also==
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*[[Antibody|Antibody]]
*[[Beta-2 microglobulin|Beta-2 microglobulin]]
*[[Beta-2 microglobulin|Beta-2 microglobulin]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:021220305</ref><references group="xtra"/><references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Bellotti, V.]]
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[[Category: Bellotti, V]]
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[[Category: Domanska, K.]]
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[[Category: Domanska, K]]
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[[Category: Marquez, J A.]]
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[[Category: Marquez, J A]]
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[[Category: Pardon, E.]]
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[[Category: Pardon, E]]
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[[Category: Srinivasan, V.]]
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[[Category: Srinivasan, V]]
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[[Category: Steyaert, J.]]
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[[Category: Steyaert, J]]
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[[Category: Vanderhaegen, S.]]
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[[Category: Vanderhaegen, S]]
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[[Category: Wyns, L.]]
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[[Category: Wyns, L]]
[[Category: Immune system]]
[[Category: Immune system]]

Revision as of 12:14, 18 December 2014

Structure of the Beta2_microglobulin involved in amyloidogenesis

2x89, resolution 2.16Å

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