View source for 4rmv

==Crystal structure of the D76H Beta-2 Microglobulin mutant==
<StructureSection load='4rmv' size='340' side='right' caption='[[4rmv]], [[Resolution|resolution]] 1.46&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[4rmv]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4RMV OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4RMV FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=ACT:ACETATE+ION'>ACT</scene>, <scene name='pdbligand=PGE:TRIETHYLENE+GLYCOL'>PGE</scene></td></tr>
<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2yxf|2yxf]], [[4fxl|4fxl]], [[4rmq|4rmq]], [[4rmr|4rmr]], [[4rms|4rms]], [[4rmt|4rmt]], [[4rmu|4rmu]], [[4rmw|4rmw]]</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=4rmv FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4rmv OCA], [http://pdbe.org/4rmv PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4rmv RCSB], [http://www.ebi.ac.uk/pdbsum/4rmv PDBsum]</span></td></tr>
</table>
== 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>  
== Function ==
[[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. 
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Bolognesi, M]]
[[Category: Ricagno, S]]
[[Category: Rosa, M de]]
[[Category: Amyloidosis]]
[[Category: Beta-sandwitch]]
[[Category: Genetic disease]]
[[Category: Immune system]]
[[Category: Immunoglobin]]
[[Category: Mhc class i]]
[[Category: Pathologic mutation]]