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==Crystal Structure of AnkB Ankyrin Repeats in Complex with AnkR/AnkB Chimeric Autoinhibition Segment==
==Crystal Structure of AnkB Ankyrin Repeats in Complex with AnkR/AnkB Chimeric Autoinhibition Segment==
<StructureSection load='5y4d' size='340' side='right'caption='[[5y4d]], [[Resolution|resolution]] 3.30&Aring;' scene=''>
<StructureSection load='5y4d' size='340' side='right'caption='[[5y4d]], [[Resolution|resolution]] 3.30&Aring;' scene=''>
Line 8: Line 7:
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=5y4d FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5y4d OCA], [https://pdbe.org/5y4d PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=5y4d RCSB], [https://www.ebi.ac.uk/pdbsum/5y4d PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=5y4d ProSAT]</span></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=5y4d FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5y4d OCA], [https://pdbe.org/5y4d PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=5y4d RCSB], [https://www.ebi.ac.uk/pdbsum/5y4d PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=5y4d ProSAT]</span></td></tr>
</table>
</table>
<scene name='76/763657/Xyz/1'>test PNG created with my Mac with  16.2.21and loaded into default Jmol on Proteopedia</scene>
== Disease ==
== Disease ==
[https://www.uniprot.org/uniprot/ANK2_HUMAN ANK2_HUMAN] Romano-Ward syndrome. Long QT syndrome 4 (LQT4) [MIM:[https://omim.org/entry/600919 600919]: A heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy. Long QT syndrome type 4 shows many atypical features compared to classical long QT syndromes, including pronounced sinus bradycardia, polyphasic T waves and atrial fibrillation. Cardiac repolarization defects may be not as severe as in classical LQT syndromes and prolonged QT interval on EKG is not a consistent feature. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:12571597</ref> <ref>PMID:15178757</ref>  
[https://www.uniprot.org/uniprot/ANK2_HUMAN ANK2_HUMAN] Romano-Ward syndrome. Long QT syndrome 4 (LQT4) [MIM:[https://omim.org/entry/600919 600919]: A heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy. Long QT syndrome type 4 shows many atypical features compared to classical long QT syndromes, including pronounced sinus bradycardia, polyphasic T waves and atrial fibrillation. Cardiac repolarization defects may be not as severe as in classical LQT syndromes and prolonged QT interval on EKG is not a consistent feature. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:12571597</ref> <ref>PMID:15178757</ref>  

Revision as of 20:43, 28 October 2024

Crystal Structure of AnkB Ankyrin Repeats in Complex with AnkR/AnkB Chimeric Autoinhibition SegmentCrystal Structure of AnkB Ankyrin Repeats in Complex with AnkR/AnkB Chimeric Autoinhibition Segment

Structural highlights

5y4d is a 1 chain structure with sequence from Homo sapiens and Mus musculus. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:X-ray diffraction, Resolution 3.3Å
Ligands:
Resources:FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT


Disease

ANK2_HUMAN Romano-Ward syndrome. Long QT syndrome 4 (LQT4) [MIM:600919: A heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy. Long QT syndrome type 4 shows many atypical features compared to classical long QT syndromes, including pronounced sinus bradycardia, polyphasic T waves and atrial fibrillation. Cardiac repolarization defects may be not as severe as in classical LQT syndromes and prolonged QT interval on EKG is not a consistent feature. Note=The disease is caused by mutations affecting the gene represented in this entry.[1] [2]

Function

ANK2_HUMAN In skeletal muscle, required for proper localization of DMD and DCTN4 and for the formation and/or stability of a special subset of microtubules associated with costameres and neuromuscular junctions (By similarity). Attaches integral membrane proteins to cytoskeletal elements. Also binds to cytoskeletal proteins. Required for coordinate assembly of Na/Ca exchanger, Na/K ATPase and InsP3 receptor at sarcoplasmic reticulum sites in cardiomyocytes. Required for the coordinated expression of the Na/K ATPase, Na/Ca exchanger and beta-2-spectrin (SPTBN1) in the inner segment of rod photoreceptors. Required for expression and targeting of SPTBN1 in neonatal cardiomyocytes and for the regulation of neonatal cardiomyocyte contraction rate.[3]

Publication Abstract from PubMed

Ankyrins together with their spectrin partners are the master organizers of micron-scale membrane domains in diverse tissues. The 24 ankyrin (ANK) repeats of ankyrins bind to numerous membrane proteins, linking them to spectrin-based cytoskeletons at specific membrane microdomains. The accessibility of the target binding groove of ANK repeats must be regulated to achieve spatially defined functions of ankyrins/target complexes in different tissues, though little is known in this regard. Here we systemically investigated the autoinhibition mechanism of ankyrin-B/G by combined biochemical, biophysical and structural biology approaches. We discovered that the entire ANK repeats are inhibited by combinatorial and quasi-independent bindings of multiple disordered segments located in the ankyrin-B/G linkers and tails, suggesting a mechanistic basis for differential regulations of membrane target bindings by ankyrins. In addition to elucidating the autoinhibition mechanisms of ankyrins, our study may also shed light on regulations on target bindings by other long repeat-containing proteins.

Autoinhibition of ankyrin-B/G membrane target bindings by intrinsically disordered segments from the tail regions.,Chen K, Li J, Wang C, Wei Z, Zhang M Elife. 2017 Aug 25;6. pii: e29150. doi: 10.7554/eLife.29150. PMID:28841137[4]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

See Also

References

  1. ↑ Mohler PJ, Schott JJ, Gramolini AO, Dilly KW, Guatimosim S, duBell WH, Song LS, Haurogne K, Kyndt F, Ali ME, Rogers TB, Lederer WJ, Escande D, Le Marec H, Bennett V. Ankyrin-B mutation causes type 4 long-QT cardiac arrhythmia and sudden cardiac death. Nature. 2003 Feb 6;421(6923):634-9. PMID:12571597 doi:10.1038/nature01335
  2. ↑ Mohler PJ, Splawski I, Napolitano C, Bottelli G, Sharpe L, Timothy K, Priori SG, Keating MT, Bennett V. A cardiac arrhythmia syndrome caused by loss of ankyrin-B function. Proc Natl Acad Sci U S A. 2004 Jun 15;101(24):9137-42. Epub 2004 Jun 3. PMID:15178757 doi:10.1073/pnas.0402546101
  3. ↑ Mohler PJ, Schott JJ, Gramolini AO, Dilly KW, Guatimosim S, duBell WH, Song LS, Haurogne K, Kyndt F, Ali ME, Rogers TB, Lederer WJ, Escande D, Le Marec H, Bennett V. Ankyrin-B mutation causes type 4 long-QT cardiac arrhythmia and sudden cardiac death. Nature. 2003 Feb 6;421(6923):634-9. PMID:12571597 doi:10.1038/nature01335
  4. ↑ Chen K, Li J, Wang C, Wei Z, Zhang M. Autoinhibition of ankyrin-B/G membrane target bindings by intrinsically disordered segments from the tail regions. Elife. 2017 Aug 25;6. pii: e29150. doi: 10.7554/eLife.29150. PMID:28841137 doi:http://dx.doi.org/10.7554/eLife.29150

5y4d, resolution 3.30Å

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OCA, Joel L. Sussman
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