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3tv8

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{{STRUCTURE_3tv8| PDB=3tv8 | SCENE= }}
 
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===Pharmacological Chaperoning in Human alpha-Galactosidase===
 
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{{ABSTRACT_PUBMED_22195554}}
 
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==Disease==
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==Pharmacological Chaperoning in Human alpha-Galactosidase==
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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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<StructureSection load='3tv8' size='340' side='right'caption='[[3tv8]], [[Resolution|resolution]] 2.64&Aring;' scene=''>
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== Structural highlights ==
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==About this Structure==
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<table><tr><td colspan='2'>[[3tv8]] 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=3TV8 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3TV8 FirstGlance]. <br>
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[[3tv8]] 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=3TV8 OCA].
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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]] 2.639&#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=2PE:NONAETHYLENE+GLYCOL'>2PE</scene>, <scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=DGJ:(2R,3S,4R,5S)-2-(HYDROXYMETHYL)PIPERIDINE-3,4,5-TRIOL'>DGJ</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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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3tv8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3tv8 OCA], [https://pdbe.org/3tv8 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3tv8 RCSB], [https://www.ebi.ac.uk/pdbsum/3tv8 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3tv8 ProSAT]</span></td></tr>
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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]
==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:022195554</ref><references group="xtra"/><references/>
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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: Rogich, J J.]]
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[[Category: Guce AI]]
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[[Category: Carbohydrate-binding protein]]
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[[Category: Rogich JJ]]
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[[Category: Glycoprotein]]
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[[Category: Glycosidase]]
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[[Category: Hydrolase]]
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[[Category: Lysosome]]
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[[Category: N-linked glycosylation]]
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[[Category: Pharmacological chaperone]]
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Current revision

Pharmacological Chaperoning in Human alpha-Galactosidase

PDB ID 3tv8

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