View source for 6csv


==The structure of the Cep63-Cep152 heterotetrameric complex==
<StructureSection load='6csv' size='340' side='right'caption='[[6csv]], [[Resolution|resolution]] 2.50&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[6csv]] is a 4 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=6CSV OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6CSV FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.5&#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=6csv FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6csv OCA], [https://pdbe.org/6csv PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6csv RCSB], [https://www.ebi.ac.uk/pdbsum/6csv PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6csv ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/CEP63_HUMAN CEP63_HUMAN] Autosomal recessive primary microcephaly. The disease is caused by mutations affecting the gene represented in this entry.[https://www.uniprot.org/uniprot/CE152_HUMAN CE152_HUMAN] Seckel syndrome;Autosomal recessive primary microcephaly. 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.
== Function ==
[https://www.uniprot.org/uniprot/CEP63_HUMAN CEP63_HUMAN] Required for normal spindle assembly. Plays a key role in mother-centriole-dependent centriole duplication; the function seems also to involve CEP152, CDK5RAP2 and WDR62 through a stepwise assembled complex at the centrosome that recruits CDK2 required for centriole duplication. Reported to be required for centrosomal recruitment of CEP152; however, this function has been questioned (PubMed:21983783, PubMed:26297806). Also recruits CDK1 to centrosomes (PubMed:21406398). Plays a role in DNA damage response. Following DNA damage, such as double-strand breaks (DSBs), is removed from centrosomes; this leads to the inactivation of spindle assembly and delay in mitotic progression (PubMed:21406398).<ref>PMID:21406398</ref> <ref>PMID:21983783</ref> <ref>PMID:26297806</ref> [https://www.uniprot.org/uniprot/CE152_HUMAN CE152_HUMAN] Necessary for centrosome duplication. Acts as a molecular scaffold facilitating the interaction of PLK4 and CENPJ, 2 molecules involved in centriole formation. Also plays a key role in deuterosome-mediated centriole amplification in multiciliated that can generate more than 100 centrioles. Overexpression of CEP152 can drive amplification of centrioles.<ref>PMID:21059844</ref> <ref>PMID:20852615</ref> <ref>PMID:21131973</ref> 
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Ahn JI]]
[[Category: Chen Y]]
[[Category: Kim TS]]
[[Category: Lee E]]
[[Category: Lee KS]]
[[Category: Park JE]]
[[Category: Zhang L]]