2bq8

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==Crystal structure of human purple acid phosphatase with an inhibitory conformation of the repression loop==
==Crystal structure of human purple acid phosphatase with an inhibitory conformation of the repression loop==
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<StructureSection load='2bq8' size='340' side='right' caption='[[2bq8]], [[Resolution|resolution]] 2.20&Aring;' scene=''>
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<StructureSection load='2bq8' size='340' side='right'caption='[[2bq8]], [[Resolution|resolution]] 2.20&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[2bq8]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2BQ8 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2BQ8 FirstGlance]. <br>
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<table><tr><td colspan='2'>[[2bq8]] is a 1 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=2BQ8 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2BQ8 FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=FE2:FE+(II)+ION'>FE2</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
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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]] 2.2&#8491;</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/Acid_phosphatase Acid phosphatase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.1.3.2 3.1.3.2] </span></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=FE2:FE+(II)+ION'>FE2</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></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=2bq8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2bq8 OCA], [http://pdbe.org/2bq8 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2bq8 RCSB], [http://www.ebi.ac.uk/pdbsum/2bq8 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2bq8 ProSAT]</span></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=2bq8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2bq8 OCA], [https://pdbe.org/2bq8 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2bq8 RCSB], [https://www.ebi.ac.uk/pdbsum/2bq8 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2bq8 ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
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[[http://www.uniprot.org/uniprot/PPA5_HUMAN PPA5_HUMAN]] Defects in ACP5 are the cause of spondyloenchondrodysplasia with immune dysregulation (SPENCDI) [MIM:[http://omim.org/entry/607944 607944]]. A disease characterized by vertebral and metaphyseal dysplasia, spasticity with cerebral calcifications, and strong predisposition to autoimmune diseases. The skeletal dysplasia is characterized by radiolucent and irregular spondylar and metaphyseal lesions that represent islands of chondroid tissue within bone. Note=ACP5 inactivating mutations result in a functional excess of phosphorylated osteopontin causing deregulation of osteopontin signaling and consequential autoimmune disease.<ref>PMID:21217755</ref> <ref>PMID:21217752</ref>
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[https://www.uniprot.org/uniprot/PPA5_HUMAN PPA5_HUMAN] Defects in ACP5 are the cause of spondyloenchondrodysplasia with immune dysregulation (SPENCDI) [MIM:[https://omim.org/entry/607944 607944]. A disease characterized by vertebral and metaphyseal dysplasia, spasticity with cerebral calcifications, and strong predisposition to autoimmune diseases. The skeletal dysplasia is characterized by radiolucent and irregular spondylar and metaphyseal lesions that represent islands of chondroid tissue within bone. Note=ACP5 inactivating mutations result in a functional excess of phosphorylated osteopontin causing deregulation of osteopontin signaling and consequential autoimmune disease.<ref>PMID:21217755</ref> <ref>PMID:21217752</ref>
== Function ==
== Function ==
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[[http://www.uniprot.org/uniprot/PPA5_HUMAN PPA5_HUMAN]] Involved in osteopontin/bone sialoprotein dephosphorylation. Its expression seems to increase in certain pathological states such as Gaucher and Hodgkin diseases, the hairy cell, the B-cell, and the T-cell leukemias.
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[https://www.uniprot.org/uniprot/PPA5_HUMAN PPA5_HUMAN] Involved in osteopontin/bone sialoprotein dephosphorylation. Its expression seems to increase in certain pathological states such as Gaucher and Hodgkin diseases, the hairy cell, the B-cell, and the T-cell leukemias.
== Evolutionary Conservation ==
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
[[Image:Consurf_key_small.gif|200px|right]]
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<jmolCheckbox>
<jmolCheckbox>
<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/bq/2bq8_consurf.spt"</scriptWhenChecked>
<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/bq/2bq8_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview03.spt</scriptWhenUnchecked>
<text>to colour the structure by Evolutionary Conservation</text>
<text>to colour the structure by Evolutionary Conservation</text>
</jmolCheckbox>
</jmolCheckbox>
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==See Also==
==See Also==
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*[[Acid phosphatase|Acid phosphatase]]
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*[[Acid phosphatase 3D structures|Acid phosphatase 3D structures]]
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Acid phosphatase]]
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[[Category: Homo sapiens]]
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[[Category: Human]]
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[[Category: Large Structures]]
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[[Category: Jasper, B]]
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[[Category: Jasper B]]
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[[Category: Krebs, B]]
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[[Category: Krebs B]]
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[[Category: Straeter, N]]
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[[Category: Straeter N]]
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[[Category: Dinuclear metal site]]
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[[Category: Hydrolase]]
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[[Category: Metallophosphatase]]
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[[Category: Trap]]
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Current revision

Crystal structure of human purple acid phosphatase with an inhibitory conformation of the repression loop

PDB ID 2bq8

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