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| ==Actinin-1 EF-Hand bound to the Cav1.2 IQ Motif== | | ==Actinin-1 EF-Hand bound to the Cav1.2 IQ Motif== |
| <StructureSection load='6c0a' size='340' side='right' caption='[[6c0a]], [[NMR_Ensembles_of_Models | 4 NMR models]]' scene=''> | | <StructureSection load='6c0a' size='340' side='right'caption='[[6c0a]]' scene=''> |
| == Structural highlights == | | == Structural highlights == |
| <table><tr><td colspan='2'>[[6c0a]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Lk3_transgenic_mice Lk3 transgenic mice]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6C0A OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6C0A FirstGlance]. <br> | | <table><tr><td colspan='2'>[[6c0a]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Mus_musculus Mus musculus]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6C0A OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6C0A FirstGlance]. <br> |
| </td></tr><tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">Actn1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=10090 LK3 transgenic mice])</td></tr> | | </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=6c0a FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6c0a OCA], [https://pdbe.org/6c0a PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6c0a RCSB], [https://www.ebi.ac.uk/pdbsum/6c0a PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6c0a ProSAT]</span></td></tr> |
| <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6c0a FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6c0a OCA], [http://pdbe.org/6c0a PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6c0a RCSB], [http://www.ebi.ac.uk/pdbsum/6c0a PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6c0a ProSAT]</span></td></tr> | |
| </table> | | </table> |
| == Disease ==
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| [[http://www.uniprot.org/uniprot/CAC1C_HUMAN CAC1C_HUMAN]] Defects in CACNA1C are the cause of Timothy syndrome (TS) [MIM:[http://omim.org/entry/601005 601005]]. TS is a disorder characterized by multiorgan dysfunction including lethal arrhythmias, webbing of fingers and toes, congenital heart disease, immune deficiency, intermittent hypoglycemia, cognitive abnormalities and autism.<ref>PMID:15454078</ref> <ref>PMID:15863612</ref> Defects in CACNA1C are the cause of Brugada syndrome type 3 (BRGDA3) [MIM:[http://omim.org/entry/611875 611875]]. A heart disease characterized by the association of Brugada syndrome with shortened QT intervals. Brugada syndrome is a tachyarrhythmia characterized by right bundle branch block and ST segment elevation on an electrocardiogram (ECG). It can cause the ventricles to beat so fast that the blood is prevented from circulating efficiently in the body. When this situation occurs (called ventricular fibrillation), the individual will faint and may die in a few minutes if the heart is not reset.<ref>PMID:17224476</ref>
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| == Function == | | == Function == |
| [[http://www.uniprot.org/uniprot/ACTN1_MOUSE ACTN1_MOUSE]] F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein (By similarity). [[http://www.uniprot.org/uniprot/CAC1C_HUMAN CAC1C_HUMAN]] Voltage-sensitive calcium channels (VSCC) mediate the entry of calcium ions into excitable cells and are also involved in a variety of calcium-dependent processes, including muscle contraction, hormone or neurotransmitter release, gene expression, cell motility, cell division and cell death. The isoform alpha-1C gives rise to L-type calcium currents. Long-lasting (L-type) calcium channels belong to the 'high-voltage activated' (HVA) group. They are blocked by dihydropyridines (DHP), phenylalkylamines, benzothiazepines, and by omega-agatoxin-IIIA (omega-Aga-IIIA). They are however insensitive to omega-conotoxin-GVIA (omega-CTx-GVIA) and omega-agatoxin-IVA (omega-Aga-IVA). Calcium channels containing the alpha-1C subunit play an important role in excitation-contraction coupling in the heart. The various isoforms display marked differences in the sensitivity to DHP compounds. Binding of calmodulin or CABP1 at the same regulatory sites results in an opposit effects on the channel function.<ref>PMID:8392192</ref> <ref>PMID:7737988</ref> <ref>PMID:9013606</ref> <ref>PMID:9607315</ref> <ref>PMID:12176756</ref> <ref>PMID:17071743</ref> | | [https://www.uniprot.org/uniprot/ACTN1_MOUSE ACTN1_MOUSE] F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein (By similarity). |
| <div style="background-color:#fffaf0;">
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| == Publication Abstract from PubMed ==
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| The regulation and localization of the neuronal voltage gated Ca(2+) channel CaV1.2 is important for synaptic plasticity associated with learning and memory. The cytoskeletal protein, alpha-actinin-1 is known to interact with CaV1.2 and stabilize its localization at the postsynaptic membrane. Here we report both backbone and sidechain NMR assignments for the C-terminal EF-hands (EF3 and EF4) of alpha-actinin-1 (residues 824-892, called ACTN_EF34) bound to the IQ-motif (residues 1644-1665) from CaV1.2 (BMRB accession no. 25902).
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| Chemical shift assignments of the C-terminal EF-hand domain of alpha-actinin-1.,Turner M, Anderson DE, Rajan S, Hell JW, Ames JB Biomol NMR Assign. 2016 Apr;10(1):219-22. doi: 10.1007/s12104-016-9670-2. Epub, 2016 Feb 10. PMID:26861220<ref>PMID:26861220</ref>
| | ==See Also== |
| | | *[[Actinin 3D structures|Actinin 3D structures]] |
| From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>
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| </div>
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| <div class="pdbe-citations 6c0a" style="background-color:#fffaf0;"></div>
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| == References == | |
| <references/>
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| __TOC__ | | __TOC__ |
| </StructureSection> | | </StructureSection> |
| [[Category: Lk3 transgenic mice]] | | [[Category: Homo sapiens]] |
| [[Category: Ames, J B]] | | [[Category: Large Structures]] |
| [[Category: Turner, M L]] | | [[Category: Mus musculus]] |
| [[Category: Actinin-1]] | | [[Category: Ames JB]] |
| [[Category: Calcium calmodulin]] | | [[Category: Turner ML]] |
| [[Category: Cav1 2]]
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| [[Category: Ef hand]]
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| [[Category: Hippocampal neuron]]
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| [[Category: Iq motif]]
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| [[Category: Neuronal signaling]]
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| [[Category: Signaling protein]]
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| [[Category: Voltage gated calcium channel]]
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| [[Category: Voltage gated calcium channel 1 2]]
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