1hyn

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(New page: 200px<br /> <applet load="1hyn" size="450" color="white" frame="true" align="right" spinBox="true" caption="1hyn, resolution 2.6&Aring;" /> '''CRYSTAL STRUCTURE OF...)
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[[Image:1hyn.gif|left|200px]]<br />
 
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<applet load="1hyn" size="450" color="white" frame="true" align="right" spinBox="true"
 
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caption="1hyn, resolution 2.6&Aring;" />
 
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'''CRYSTAL STRUCTURE OF THE CYTOPLASMIC DOMAIN OF HUMAN ERYTHROCYTE BAND-3 PROTEIN'''<br />
 
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==Overview==
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==CRYSTAL STRUCTURE OF THE CYTOPLASMIC DOMAIN OF HUMAN ERYTHROCYTE BAND-3 PROTEIN==
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The red blood cell membrane (RBCM) is a primary model for animal cell, plasma membranes. One of its major organizing centers is the cytoplasmic, domain of band 3 (cdb3), which links multiple proteins to the membrane., Included among its peripheral protein ligands are ankyrin (the major, bridge to the spectrin-actin skeleton), protein 4. 1, protein 4.2, aldolase, glyceraldehyde-3-phosphate dehydrogenase, phosphofructokinase, deoxyhemoglobin, p72syk protein tyrosine kinase, and hemichromes. The, crystal structure of cdb3 is reported at 0.26 nm (2.6 A) resolution. A, tight symmetric dimer is formed by cdb3; it is stabilized by interlocked, dimerization arms contributed by both monomers. Each subunit also includes, a larger peripheral protein binding domain with an alpha(+) beta-fold. The, binding sites of several peripheral proteins are localized in the, structure, and the nature of the major conformational change that, regulates membrane-skeletal interactions is evaluated. An improved, structural definition of the protein network at the inner surface of the, RBCM is now possible.
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<StructureSection load='1hyn' size='340' side='right'caption='[[1hyn]], [[Resolution|resolution]] 2.60&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[1hyn]] is a 4 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=1HYN OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1HYN FirstGlance]. <br>
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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]] 2.6&#8491;</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=1hyn FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1hyn OCA], [https://pdbe.org/1hyn PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1hyn RCSB], [https://www.ebi.ac.uk/pdbsum/1hyn PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1hyn ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/B3AT_HUMAN B3AT_HUMAN] Defects in SLC4A1 are the cause of elliptocytosis type 4 (EL4) [MIM:[https://omim.org/entry/109270 109270]. EL4 is a Rhesus-unlinked form of hereditary elliptocytosis, a genetically heterogeneous, autosomal dominant hematologic disorder. It is characterized by variable hemolytic anemia and elliptical or oval red cell shape.<ref>PMID:1722314</ref> <ref>PMID:1538405</ref> Defects in SLC4A1 are the cause of spherocytosis type 4 (SPH4) [MIM:[https://omim.org/entry/612653 612653]; also known as hereditary spherocytosis type 4 (HS4). Spherocytosis is a hematologic disorder leading to chronic hemolytic anemia and characterized by numerous abnormally shaped erythrocytes which are generally spheroidal.<ref>PMID:8547122</ref> <ref>PMID:1378323</ref> <ref>PMID:7530501</ref> <ref>PMID:8943874</ref> <ref>PMID:8640229</ref> <ref>PMID:9207478</ref> <ref>PMID:9012689</ref> <ref>PMID:9233560</ref> <ref>PMID:9973643</ref> <ref>PMID:10580570</ref> <ref>PMID:10942416</ref> <ref>PMID:10745622</ref> <ref>PMID:11380459</ref> <ref>PMID:15813913</ref> <ref>PMID:16227998</ref> Defects in SLC4A1 are the cause of renal tubular acidosis, distal, autosomal dominant (AD-dRTA) [MIM:[https://omim.org/entry/179800 179800]. A disease characterized by reduced ability to acidify urine, variable hyperchloremic hypokalemic metabolic acidosis, nephrocalcinosis, and nephrolithiasis. Defects in SLC4A1 are the cause of renal tubular acidosis, distal, with hemolytic anemia (dRTA-HA) [MIM:[https://omim.org/entry/611590 611590]. A disease characterized by the association of hemolytic anemia with distal renal tubular acidosis, the reduced ability to acidify urine resulting in variable hyperchloremic hypokalemic metabolic acidosis, nephrocalcinosis, and nephrolithiasis. Defects in SLC4A1 are the cause of renal tubular acidosis, distal, with normal red cell morphology (dRTA-NRC) [MIM:[https://omim.org/entry/611590 611590]. A disease characterized by reduced ability to acidify urine, variable hyperchloremic hypokalemic metabolic acidosis, nephrocalcinosis, and nephrolithiasis.
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== Function ==
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[https://www.uniprot.org/uniprot/B3AT_HUMAN B3AT_HUMAN] Band 3 is the major integral glycoprotein of the erythrocyte membrane. Band 3 has two functional domains. Its integral domain mediates a 1:1 exchange of inorganic anions across the membrane, whereas its cytoplasmic domain provides binding sites for cytoskeletal proteins, glycolytic enzymes, and hemoglobin.
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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/hy/1hyn_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=1hyn ConSurf].
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<div style="clear:both"></div>
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==Disease==
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==See Also==
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Known diseases associated with this structure: Acanthocytosis, one form OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=109270 109270]], Blood group, Diego OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=109270 109270]], Blood group, Waldner OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=109270 109270]], Blood group, Wright OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=109270 109270]], Diabetes insipidus, nephrogenic OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=300538 300538]], Elliptocytosis, Malaysian-Melanesian type OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=109270 109270]], Hemolytic anemia due to band 3 defect defect OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=109270 109270]], Nephrogenic syndrome of inappropriate antidiuresis OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=300538 300538]], OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id= ]]109270, Renal tubular acidosis, distal, 179800 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=109270 109270]], Spherocytosis, hereditary OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=109270 109270]]
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*[[Anion exchange protein 3D structures|Anion exchange protein 3D structures]]
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== References ==
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==About this Structure==
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<references/>
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1HYN is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=1HYN OCA].
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__TOC__
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</StructureSection>
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==Reference==
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Crystallographic structure and functional interpretation of the cytoplasmic domain of erythrocyte membrane band 3., Zhang D, Kiyatkin A, Bolin JT, Low PS, Blood. 2000 Nov 1;96(9):2925-33. PMID:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=11049968 11049968]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Bolin, J.T.]]
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[[Category: Bolin JT]]
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[[Category: Kiyatkin, A.]]
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[[Category: Kiyatkin A]]
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[[Category: Low, P.S.]]
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[[Category: Low PS]]
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[[Category: Zhang, D.]]
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[[Category: Zhang D]]
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[[Category: membrane protein]]
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''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 17:24:18 2007''
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Current revision

CRYSTAL STRUCTURE OF THE CYTOPLASMIC DOMAIN OF HUMAN ERYTHROCYTE BAND-3 PROTEIN

PDB ID 1hyn

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