View source for 23ph


==Cryo-EM structures of the human ABCB7 in the apo state==
<StructureSection load='23ph' size='340' side='right'caption='[[23ph]], [[Resolution|resolution]] 3.01&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[23ph]] 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=23PH OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=23PH FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3.01&#8491;</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=23ph FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=23ph OCA], [https://pdbe.org/23ph PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=23ph RCSB], [https://www.ebi.ac.uk/pdbsum/23ph PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=23ph ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/ABCB7_HUMAN ABCB7_HUMAN] X-linked sideroblastic anemia and spinocerebellar ataxia. The disease is caused by variants affecting the gene represented in this entry.
== Function ==
[https://www.uniprot.org/uniprot/ABCB7_HUMAN ABCB7_HUMAN] Exports glutathione-coordinated iron-sulfur clusters such as [2Fe-2S]-(GS)4 cluster from the mitochondria to the cytosol in an ATP-dependent manner allowing the assembly of the cytosolic iron-sulfur (Fe/S) cluster-containing proteins and participates in iron homeostasis (PubMed:10196363, PubMed:17192393, PubMed:33157103). Moreover, through a functional complex formed of ABCB7, FECH and ABCB10, also plays a role in the cellular iron homeostasis, mitochondrial function and heme biosynthesis (PubMed:30765471). In cardiomyocytes, regulates cellular iron homeostasis and cellular reactive oxygen species (ROS) levels through its interaction with COX4I1 (By similarity). May also play a role in hematopoiesis (By similarity).[UniProtKB:Q61102][UniProtKB:Q704E8]<ref>PMID:10196363</ref> <ref>PMID:17192393</ref> <ref>PMID:30765471</ref> <ref>PMID:33157103</ref> 
== References ==
<references/>
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Choi SH]]
[[Category: Jin MS]]
[[Category: Ju S]]
[[Category: Lee HY]]