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{{STRUCTURE_3pyk|  PDB=3pyk  |  SCENE=  }}
===Human Carbonic Anhydrase II as Host for Pianostool Complexes Bearing a Sulfonamide Anchor===
{{ABSTRACT_PUBMED_21706094}}


==Disease==
==Human Carbonic Anhydrase II as Host for Pianostool Complexes Bearing a Sulfonamide Anchor==
[[http://www.uniprot.org/uniprot/CAH2_HUMAN CAH2_HUMAN]] Defects in CA2 are the cause of osteopetrosis autosomal recessive type 3 (OPTB3) [MIM:[http://omim.org/entry/259730 259730]]; also known as osteopetrosis with renal tubular acidosis, carbonic anhydrase II deficiency syndrome, Guibaud-Vainsel syndrome or marble brain disease. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. The disorder occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or adulthood. Autosomal recessive osteopetrosis is usually associated with normal or elevated amount of non-functional osteoclasts. OPTB3 is associated with renal tubular acidosis, cerebral calcification (marble brain disease) and in some cases with mental retardation.<ref>PMID:1928091</ref><ref>PMID:1542674</ref><ref>PMID:8834238</ref><ref>PMID:9143915</ref><ref>PMID:15300855</ref>  
<StructureSection load='3pyk' size='340' side='right'caption='[[3pyk]], [[Resolution|resolution]] 1.30&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[3pyk]] 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=3PYK OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3PYK FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.3&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=MMC:METHYL+MERCURY+ION'>MMC</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=SRX:CHLORO{N-[DI(PYRIDIN-2-YL-KAPPAN)METHYL]-4-SULFAMOYLBENZAMIDE}[(1,2,3,4,5,6-ETA)-(1R,2R,3R,4S,5S,6S)-1,2,3,4,5,6-HEXAMETHYLCYCLOHEXANE-1,2,3,4,5,6-HEXAYL]RUTHENIUM(2+)'>SRX</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3pyk FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3pyk OCA], [https://pdbe.org/3pyk PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3pyk RCSB], [https://www.ebi.ac.uk/pdbsum/3pyk PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3pyk ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/CAH2_HUMAN CAH2_HUMAN] Defects in CA2 are the cause of osteopetrosis autosomal recessive type 3 (OPTB3) [MIM:[https://omim.org/entry/259730 259730]; also known as osteopetrosis with renal tubular acidosis, carbonic anhydrase II deficiency syndrome, Guibaud-Vainsel syndrome or marble brain disease. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. The disorder occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or adulthood. Autosomal recessive osteopetrosis is usually associated with normal or elevated amount of non-functional osteoclasts. OPTB3 is associated with renal tubular acidosis, cerebral calcification (marble brain disease) and in some cases with mental retardation.<ref>PMID:1928091</ref> <ref>PMID:1542674</ref> <ref>PMID:8834238</ref> <ref>PMID:9143915</ref> <ref>PMID:15300855</ref>  
== Function ==
[https://www.uniprot.org/uniprot/CAH2_HUMAN CAH2_HUMAN] Essential for bone resorption and osteoclast differentiation (By similarity). Reversible hydration of carbon dioxide. Can hydrate cyanamide to urea. Involved in the regulation of fluid secretion into the anterior chamber of the eye.<ref>PMID:10550681</ref> <ref>PMID:11831900</ref>
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
d(6)-piano-stool complexes bearing an arylsulfonamide anchor display sub-micromolar affinity towards human Carbonic Anhydrase II (hCA II). The 1.3 A resolution X-ray crystal structure of [(eta(6)-C(6)Me(6))Ru(bispy 3)Cl](+) subset hCA II highlights the nature of the host-guest interactions.


==Function==
Human Carbonic Anhydrase II as a host for piano-stool complexes bearing a sulfonamide anchor.,Monnard FW, Heinisch T, Nogueira ES, Schirmer T, Ward TR Chem Commun (Camb). 2011 Aug 7;47(29):8238-40. Epub 2011 Jun 27. PMID:21706094<ref>PMID:21706094</ref>
[[http://www.uniprot.org/uniprot/CAH2_HUMAN CAH2_HUMAN]] Essential for bone resorption and osteoclast differentiation (By similarity). Reversible hydration of carbon dioxide. Can hydrate cyanamide to urea. Involved in the regulation of fluid secretion into the anterior chamber of the eye.<ref>PMID:10550681</ref><ref>PMID:11831900</ref>  


==About this Structure==
From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
[[3pyk]] is a 1 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=3PYK OCA].
</div>
<div class="pdbe-citations 3pyk" style="background-color:#fffaf0;"></div>


==See Also==
==See Also==
*[[Carbonic anhydrase|Carbonic anhydrase]]
*[[Carbonic anhydrase 3D structures|Carbonic anhydrase 3D structures]]
 
== References ==
==Reference==
<references/>
<ref group="xtra">PMID:021706094</ref><references group="xtra"/><references/>
__TOC__
[[Category: Carbonate dehydratase]]
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Heinisch, T.]]
[[Category: Large Structures]]
[[Category: Schirmer, T.]]
[[Category: Heinisch T]]
[[Category: 10 stranded]]
[[Category: Schirmer T]]
[[Category: Bicarbonate]]
[[Category: Carbonate dehydratase activity]]
[[Category: Co2]]
[[Category: Lyase activity]]
[[Category: Lyase-lyase inhibitor complex]]
[[Category: Metal ion binding]]
[[Category: Metal ion]]
[[Category: Protein binding]]
[[Category: Sulfonamide]]
[[Category: Twisted beta-sheet]]
[[Category: Zinc ion binding]]
Retrieved from "https://proteopedia.org/3pyk"