8yr3: Difference between revisions

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'''Unreleased structure'''


The entry 8yr3 is ON HOLD  until Paper Publication
==Cryo-EM structure of the human ABCB6 in complex with Cd(II):GSH==
 
<StructureSection load='8yr3' size='340' side='right'caption='[[8yr3]], [[Resolution|resolution]] 3.20&Aring;' scene=''>
Authors: Choi, S.H., Lee, S.S., Lee, H.Y., Kim, S., Kim, J.W., Jin, M.S.
== Structural highlights ==
 
<table><tr><td colspan='2'>[[8yr3]] 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=8YR3 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8YR3 FirstGlance]. <br>
Description: Cryo-EM structure of the human ABCB6 in complex with Cd(II):GSH
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3.2&#8491;</td></tr>
[[Category: Unreleased Structures]]
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CD:CADMIUM+ION'>CD</scene>, <scene name='pdbligand=GSH:GLUTATHIONE'>GSH</scene></td></tr>
[[Category: Kim, J.W]]
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=8yr3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8yr3 OCA], [https://pdbe.org/8yr3 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8yr3 RCSB], [https://www.ebi.ac.uk/pdbsum/8yr3 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8yr3 ProSAT]</span></td></tr>
[[Category: Jin, M.S]]
</table>
[[Category: Lee, H.Y]]
== Disease ==
[[Category: Kim, S]]
[https://www.uniprot.org/uniprot/ABCB6_HUMAN ABCB6_HUMAN] Ocular coloboma;Dyschromatosis universalis;Colobomatous microphthalmia. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. ABCB6 mutations are involved in familial pseudohyperkalemia, a dominantly inherited condition characterized by increased serum potassium levels, measured in whole-blood specimens stored at or below room temperature. This condition is not accompanied by clinical symptoms or biological signs except for borderline abnormalities of red cell shape (PubMed:23180570).<ref>PMID:23180570</ref>
[[Category: Lee, S.S]]
== Function ==
[[Category: Choi, S.H]]
[https://www.uniprot.org/uniprot/ABCB6_HUMAN ABCB6_HUMAN] Binds heme and porphyrins and functions in their ATP-dependent uptake into the mitochondria. Plays a crucial role in heme synthesis.<ref>PMID:10837493</ref> <ref>PMID:17006453</ref>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Choi SH]]
[[Category: Jin MS]]
[[Category: Kim JW]]
[[Category: Kim S]]
[[Category: Lee HY]]
[[Category: Lee SS]]

Latest revision as of 08:57, 19 June 2024

Cryo-EM structure of the human ABCB6 in complex with Cd(II):GSHCryo-EM structure of the human ABCB6 in complex with Cd(II):GSH

Structural highlights

8yr3 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:Electron Microscopy, Resolution 3.2Å
Ligands:,
Resources:FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

ABCB6_HUMAN Ocular coloboma;Dyschromatosis universalis;Colobomatous microphthalmia. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. ABCB6 mutations are involved in familial pseudohyperkalemia, a dominantly inherited condition characterized by increased serum potassium levels, measured in whole-blood specimens stored at or below room temperature. This condition is not accompanied by clinical symptoms or biological signs except for borderline abnormalities of red cell shape (PubMed:23180570).[1]

Function

ABCB6_HUMAN Binds heme and porphyrins and functions in their ATP-dependent uptake into the mitochondria. Plays a crucial role in heme synthesis.[2] [3]

References

  1. ↑ Andolfo I, Alper SL, Delaunay J, Auriemma C, Russo R, Asci R, Esposito MR, Sharma AK, Shmukler BE, Brugnara C, De Franceschi L, Iolascon A. Missense mutations in the ABCB6 transporter cause dominant familial pseudohyperkalemia. Am J Hematol. 2013 Jan;88(1):66-72. doi: 10.1002/ajh.23357. Epub 2012 Nov 24. PMID:23180570 doi:http://dx.doi.org/10.1002/ajh.23357
  2. ↑ Mitsuhashi N, Miki T, Senbongi H, Yokoi N, Yano H, Miyazaki M, Nakajima N, Iwanaga T, Yokoyama Y, Shibata T, Seino S. MTABC3, a novel mitochondrial ATP-binding cassette protein involved in iron homeostasis. J Biol Chem. 2000 Jun 9;275(23):17536-40. PMID:10837493
  3. ↑ Krishnamurthy PC, Du G, Fukuda Y, Sun D, Sampath J, Mercer KE, Wang J, Sosa-Pineda B, Murti KG, Schuetz JD. Identification of a mammalian mitochondrial porphyrin transporter. Nature. 2006 Oct 5;443(7111):586-9. Epub 2006 Sep 27. PMID:17006453 doi:http://dx.doi.org/10.1038/nature05125

8yr3, resolution 3.20Å

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