6ibm

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'''Unreleased structure'''
 
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The entry 6ibm is ON HOLD until Paper Publication
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==Crystal structure of human alpha-galactosidase A in complex with alpha-galactose configured cyclosulfate ME776==
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<StructureSection load='6ibm' size='340' side='right'caption='[[6ibm]], [[Resolution|resolution]] 2.07&Aring;' scene=''>
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Authors: Rowland, R.J., Wu, L., Davies, G.J.
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== Structural highlights ==
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<table><tr><td colspan='2'>[[6ibm]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6IBM OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6IBM FirstGlance]. <br>
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Description: Crystal structure of human alpha-galactosidase A in complex with alpha-galactose configured cyclosulfate ME776
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</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=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=HF8:[(1~{S},2~{R},3~{S},4~{S},5~{R},6~{S})-2-(hydroxymethyl)-3,4,5,6-tetrakis(oxidanyl)cyclohexyl]+hydrogen+sulfate'>HF8</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
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[[Category: Unreleased Structures]]
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[6ibk|6ibk]]</td></tr>
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<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Alpha-galactosidase Alpha-galactosidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.22 3.2.1.22] </span></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=6ibm FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6ibm OCA], [http://pdbe.org/6ibm PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6ibm RCSB], [http://www.ebi.ac.uk/pdbsum/6ibm PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6ibm ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[[http://www.uniprot.org/uniprot/AGAL_HUMAN AGAL_HUMAN]] Defects in GLA are the cause of Fabry disease (FD) [MIM:[http://omim.org/entry/301500 301500]]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.<ref>PMID:2152885</ref> <ref>PMID:1846223</ref> <ref>PMID:2171331</ref> <ref>PMID:2539398</ref> <ref>PMID:1315715</ref> <ref>PMID:7504405</ref> <ref>PMID:8395937</ref> <ref>PMID:8069316</ref> <ref>PMID:7531540</ref> <ref>PMID:7575533</ref> <ref>PMID:7759078</ref> <ref>PMID:7599642</ref> <ref>PMID:7596372</ref> <ref>PMID:8738659</ref> <ref>PMID:8875188</ref> <ref>PMID:8834244</ref> <ref>PMID:8931708</ref> <ref>PMID:8807334</ref> <ref>PMID:8863162</ref> <ref>PMID:9105656</ref> <ref>PMID:9100224</ref> <ref>PMID:9554750</ref> <ref>PMID:9452068</ref> <ref>PMID:9452090</ref> <ref>PMID:9452111</ref> <ref>PMID:10208848</ref> <ref>PMID:10090526</ref> <ref>PMID:10838196</ref> <ref>PMID:10666480</ref> <ref>PMID:11076046</ref> <ref>PMID:10916280</ref> <ref>PMID:11295840</ref> <ref>PMID:11668641</ref> <ref>PMID:11889412</ref> <ref>PMID:12694230</ref> <ref>PMID:12786754</ref> <ref>PMID:15162124</ref> <ref>PMID:15712228</ref> <ref>PMID:16533976</ref> <ref>PMID:19621417</ref>
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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: Alpha-galactosidase]]
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[[Category: Large Structures]]
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[[Category: Davies, G J]]
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[[Category: Rowland, R J]]
[[Category: Wu, L]]
[[Category: Wu, L]]
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[[Category: Rowland, R.J]]
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[[Category: Carbohydrate]]
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[[Category: Davies, G.J]]
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[[Category: Galactose]]
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[[Category: Glycosidase]]
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[[Category: Hydrolase]]

Revision as of 05:16, 10 October 2019

Crystal structure of human alpha-galactosidase A in complex with alpha-galactose configured cyclosulfate ME776

PDB ID 6ibm

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