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3hg4

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[[Image:3hg4.png|left|200px]]
 
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{{STRUCTURE_3hg4| PDB=3hg4 | SCENE= }}
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==Human alpha-galactosidase catalytic mechanism 3. Covalent intermediate==
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<StructureSection load='3hg4' size='340' side='right'caption='[[3hg4]], [[Resolution|resolution]] 2.30&Aring;' scene=''>
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===Human alpha-galactosidase catalytic mechanism 3. Covalent intermediate===
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== Structural highlights ==
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<table><tr><td colspan='2'>[[3hg4]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3HG4 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3HG4 FirstGlance]. <br>
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{{ABSTRACT_PUBMED_19940122}}
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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]] 2.3&#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=7JZ:2-DEOXY-2,2-DIFLUORO-BETA-D-LYXO-HEXOPYRANOSE'>7JZ</scene>, <scene name='pdbligand=ACY:ACETIC+ACID'>ACY</scene>, <scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=FUC:ALPHA-L-FUCOSE'>FUC</scene>, <scene name='pdbligand=GAL:BETA-D-GALACTOSE'>GAL</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
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==About this Structure==
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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=3hg4 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3hg4 OCA], [https://pdbe.org/3hg4 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3hg4 RCSB], [https://www.ebi.ac.uk/pdbsum/3hg4 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3hg4 ProSAT]</span></td></tr>
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[[3hg4]] is a 2 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=3HG4 OCA].
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/AGAL_HUMAN AGAL_HUMAN] Defects in GLA are the cause of Fabry disease (FD) [MIM:[https://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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== Function ==
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[https://www.uniprot.org/uniprot/AGAL_HUMAN AGAL_HUMAN]
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/hg/3hg4_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=3hg4 ConSurf].
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<div style="clear:both"></div>
==See Also==
==See Also==
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*[[Galactosidase|Galactosidase]]
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*[[Galactosidase 3D structures|Galactosidase 3D structures]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:019940122</ref><references group="xtra"/>
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__TOC__
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[[Category: Alpha-galactosidase]]
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Clark, N E.]]
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[[Category: Large Structures]]
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[[Category: Garman, S C.]]
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[[Category: Clark NE]]
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[[Category: Guce, A I.]]
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[[Category: Garman SC]]
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[[Category: Carbohydrate-binding protein]]
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[[Category: Guce AI]]
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[[Category: Disease mutation]]
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[[Category: Disulfide bond]]
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[[Category: Glycoprotein]]
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[[Category: Glycosidase]]
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[[Category: Hydrolase]]
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[[Category: Lysosomal enzyme]]
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[[Category: Lysosome]]
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Current revision

Human alpha-galactosidase catalytic mechanism 3. Covalent intermediate

PDB ID 3hg4

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