View source for 7qj6


==Structure of recombinant human gamma-Tubulin Ring Complex 10-spoked assembly intermediate (spokes 3-12, substoichiometric spokes 13-14)==
<StructureSection load='7qj6' size='340' side='right'caption='[[7qj6]], [[Resolution|resolution]] 7.80&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[7qj6]] is a 31 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=7QJ6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7QJ6 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]] 7.8&#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=7qj6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7qj6 OCA], [https://pdbe.org/7qj6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7qj6 RCSB], [https://www.ebi.ac.uk/pdbsum/7qj6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7qj6 ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/ACTB_HUMAN ACTB_HUMAN] Defects in ACTB are a cause of dystonia juvenile-onset (DYTJ) [MIM:[https://omim.org/entry/607371 607371]. DYTJ is a form of dystonia with juvenile onset. Dystonia is defined by the presence of sustained involuntary muscle contraction, often leading to abnormal postures. DYTJ patients manifest progressive, generalized, dopa-unresponsive dystonia, developmental malformations and sensory hearing loss.<ref>PMID:16685646</ref>   Defects in ACTB are the cause of Baraitser-Winter syndrome type 1 (BRWS1) [MIM:[https://omim.org/entry/243310 243310]. A rare developmental disorder characterized by the combination of congenital ptosis, high-arched eyebrows, hypertelorism, ocular colobomata, and a brain malformation consisting of anterior-predominant lissencephaly. Other typical features include postnatal short stature and microcephaly, intellectual disability, seizures, and hearing loss.<ref>PMID:22366783</ref> 
== Function ==
[https://www.uniprot.org/uniprot/ACTB_HUMAN ACTB_HUMAN] Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells.
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== Publication Abstract from PubMed ==
The gamma-tubulin ring complex (gamma-TuRC) is the principal microtubule nucleation template in vertebrates. Recent cryo-EM reconstructions visualized the intricate quaternary structure of the gamma-TuRC, containing more than thirty subunits, raising fundamental questions about gamma-TuRC assembly and the role of actin as an integral part of the complex. Here, we reveal the structural mechanism underlying modular gamma-TuRC assembly and identify a functional role of actin in microtubule nucleation. During gamma-TuRC assembly, a GCP6-stabilized core comprising GCP2-3-4-5-4-6 is expanded by stepwise recruitment, selective stabilization and conformational locking of four pre-formed GCP2-GCP3 units. Formation of the lumenal bridge specifies incorporation of the terminal GCP2-GCP3 unit and thereby leads to closure of the gamma-TuRC ring in a left-handed spiral configuration. Actin incorporation into the complex is not relevant for gamma-TuRC assembly and structural integrity, but determines gamma-TuRC geometry and is required for efficient microtubule nucleation and mitotic chromosome alignment in vivo.

Modular assembly of the principal microtubule nucleator gamma-TuRC.,Wurtz M, Zupa E, Atorino ES, Neuner A, Bohler A, Rahadian AS, Vermeulen BJA, Tonon G, Eustermann S, Schiebel E, Pfeffer S Nat Commun. 2022 Jan 25;13(1):473. doi: 10.1038/s41467-022-28079-0. PMID:35078983<ref>PMID:35078983</ref>

From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 7qj6" style="background-color:#fffaf0;"></div>

==See Also==
*[[Tubulin 3D Structures|Tubulin 3D Structures]]
== References ==
<references/>
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Pfeffer S]]
[[Category: Zupa E]]