View source for 6pen


==Structure of Spastin Hexamer (whole model) in complex with substrate peptide==
<SX load='6pen' size='340' side='right' viewer='molstar' caption='[[6pen]], [[Resolution|resolution]] 4.20&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[6pen]] is a 7 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Synthetic_construct Synthetic construct]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6PEN OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6PEN 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]] 4.2&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ADP:ADENOSINE-5-DIPHOSPHATE'>ADP</scene>, <scene name='pdbligand=BEF:BERYLLIUM+TRIFLUORIDE+ION'>BEF</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene></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=6pen FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6pen OCA], [https://pdbe.org/6pen PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6pen RCSB], [https://www.ebi.ac.uk/pdbsum/6pen PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6pen ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/SPAST_HUMAN SPAST_HUMAN] Defects in SPAST are the cause of spastic paraplegia autosomal dominant type 4 (SPG4) [MIM:[https://omim.org/entry/182601 182601]. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG4 is the most common form of autosomal dominant spastic paraplegias.<ref>PMID:11809724</ref> <ref>PMID:15716377</ref> <ref>PMID:17389232</ref> <ref>PMID:19000169</ref> <ref>PMID:16339213</ref> <ref>PMID:15891913</ref> <ref>PMID:10610178</ref> <ref>PMID:11039577</ref> <ref>PMID:10699187</ref> <ref>PMID:11015453</ref> <ref>PMID:11087788</ref> <ref>PMID:11309678</ref> <ref>PMID:12460147</ref> <ref>PMID:11843700</ref> <ref>PMID:12124993</ref> <ref>PMID:12161613</ref> <ref>PMID:11985387</ref> <ref>PMID:12163196</ref> <ref>PMID:12202986</ref> <ref>PMID:12552568</ref> <ref>PMID:12939659</ref> <ref>PMID:14732620</ref> <ref>PMID:15210521</ref> <ref>PMID:15248095</ref> <ref>PMID:15482961</ref> <ref>PMID:15159500</ref> <ref>PMID:15326248</ref> <ref>PMID:16682546</ref> <ref>PMID:16684598</ref> <ref>PMID:17594340</ref> <ref>PMID:20214791</ref> <ref>PMID:20932283</ref> <ref>PMID:20562464</ref> <ref>PMID:20718791</ref> <ref>PMID:20550563</ref> 
== Function ==
[https://www.uniprot.org/uniprot/SPAST_HUMAN SPAST_HUMAN] ATP-dependent microtubule severing protein. Microtubule severing may promote reorganization of cellular microtubule arrays and the release of microtubules from the centrosome following nucleation. Required for membrane traffic from the endoplasmic reticulum (ER) to the Golgi and for completion of the abscission stage of cytokinesis. May also play a role in axon growth and the formation of axonal branches.<ref>PMID:11809724</ref> <ref>PMID:12676568</ref> <ref>PMID:15716377</ref> <ref>PMID:16219033</ref> <ref>PMID:17389232</ref> <ref>PMID:19000169</ref> 
== References ==
<references/>
__TOC__
</SX>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Synthetic construct]]
[[Category: Han H]]
[[Category: Hill CP]]
[[Category: McCullough J]]
[[Category: Monroe N]]
[[Category: Schubert HL]]
[[Category: Sundquist WI]]