9b4p

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Current revision (06:29, 18 December 2024) (edit) (undo)
 
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'''Unreleased structure'''
 
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The entry 9b4p is ON HOLD until Paper Publication
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==Tetramer Formation of the BCL11A ZF0 Domain==
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<StructureSection load='9b4p' size='340' side='right'caption='[[9b4p]], [[Resolution|resolution]] 2.56&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[9b4p]] is a 10 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=9B4P OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=9B4P 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]] 2.56&#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=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'>[https://proteopedia.org/fgij/fg.htm?mol=9b4p FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=9b4p OCA], [https://pdbe.org/9b4p PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=9b4p RCSB], [https://www.ebi.ac.uk/pdbsum/9b4p PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=9b4p ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/BC11A_HUMAN BC11A_HUMAN] Hereditary persistence of fetal hemoglobin - beta-thalassemia. Chromosomal aberrations involving BCL11A may be a cause of lymphoid malignancies. Translocation t(2;14)(p13;q32.3) causes BCL11A deregulation and amplification.<ref>PMID:11719382</ref> The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:27453576</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/BC11A_HUMAN BC11A_HUMAN] Transcription factor associated with the BAF SWI/SNF chromatin remodeling complex (By similarity). Repressor of fetal hemoglobin (HbF) level (PubMed:26375765). Involved in brain development (PubMed:27453576). Functions as a myeloid and B-cell proto-oncogene. May play important roles in leukemogenesis and hematopoiesis. Essential factor in lymphopoiesis required for B-cell formation in fetal liver. May function as a modulator of the transcriptional repression activity of ARP1 (By similarity).[UniProtKB:Q9QYE3]<ref>PMID:26375765</ref> <ref>PMID:27453576</ref>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Down-regulation of BCL11A protein reverses the fetal (HbF, alpha(2)gamma(2)) to adult (HbA, alpha(2)beta(2)) hemoglobin switch and is exploited in gene-based therapy for hemoglobin disorders. Because of reliance on ex vivo cell manipulation and marrow transplant, such therapies cannot lessen disease burden. To develop new small-molecule approaches, we investigated the state of BCL11A protein in erythroid cells. We report that tetramer formation mediated by a single zinc finger (ZnF0) is required for production of steady-state protein. Beyond its role in protein stability, the tetramer state is necessary for gamma-globin gene repression, because an engineered monomer fails to engage a critical co-repressor complex. These aspects of BCL11A protein production identify tetramer formation as a vulnerability for HbF silencing and provide opportunities for drug discovery.
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Authors:
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A tetramer of BCL11A is required for stable protein production and fetal hemoglobin silencing.,Zheng G, Yin M, Mehta S, Chu IT, Wang S, AlShaye A, Drainville K, Buyanbat A, Bienfait F, Tenglin K, Zhu Q, Orkin SH Science. 2024 Nov 29;386(6725):1010-1018. doi: 10.1126/science.adp3025. Epub 2024 , Nov 28. PMID:39607926<ref>PMID:39607926</ref>
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Description:
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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[[Category: Unreleased Structures]]
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</div>
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<div class="pdbe-citations 9b4p" style="background-color:#fffaf0;"></div>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
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[[Category: Homo sapiens]]
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[[Category: Large Structures]]
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[[Category: Orkin SH]]
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[[Category: Tenglin K]]
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[[Category: Yin M]]

Current revision

Tetramer Formation of the BCL11A ZF0 Domain

PDB ID 9b4p

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Proteopedia Page Contributors and Editors (what is this?)

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