4idq

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[[Image:4idq.png|left|200px]]
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==human atlastin-1 1-446, N440T, GDPAlF4-==
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<StructureSection load='4idq' size='340' side='right' caption='[[4idq]], [[Resolution|resolution]] 2.29&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[4idq]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4IDQ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4IDQ FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=ALF:TETRAFLUOROALUMINATE+ION'>ALF</scene>, <scene name='pdbligand=GDP:GUANOSINE-5-DIPHOSPHATE'>GDP</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=P6G:HEXAETHYLENE+GLYCOL'>P6G</scene></td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[4idn|4idn]], [[4ido|4ido]], [[4idp|4idp]], [[3q5d|3q5d]], [[3q5e|3q5e]]</td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">ATL1, GBP3, SPG3A ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
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<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=4idq FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4idq OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4idq RCSB], [http://www.ebi.ac.uk/pdbsum/4idq PDBsum]</span></td></tr>
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</table>
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== Disease ==
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[[http://www.uniprot.org/uniprot/ATLA1_HUMAN ATLA1_HUMAN]] Hereditary sensory and autonomic neuropathy type 1;Autosomal dominant spastic paraplegia type 3. Spastic paraplegia autosomal dominant 3 (SPG3) [MIM:[http://omim.org/entry/182600 182600]]: A form of spastic paraplegia, 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. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:17321752</ref> <ref>PMID:11685207</ref> <ref>PMID:12112092</ref> <ref>PMID:12939451</ref> <ref>PMID:14695538</ref> <ref>PMID:15184642</ref> <ref>PMID:16533974</ref> <ref>PMID:17427918</ref> <ref>PMID:20932283</ref> <ref>PMID:20718791</ref> Hereditary sensory neuropathy 1D (HSN1D) [MIM:[http://omim.org/entry/613708 613708]]: A disease characterized by adult-onset distal axonal sensory neuropathy leading to mutilating ulcerations as well as hyporeflexia. Some patients may show features suggesting upper neuron involvement. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:21194679</ref>
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== Function ==
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[[http://www.uniprot.org/uniprot/ATLA1_HUMAN ATLA1_HUMAN]] GTPase tethering membranes through formation of trans-homooligomer and mediating homotypic fusion of endoplasmic reticulum membranes. Functions in endoplasmic reticulum tubular network biogenesis. May also regulate Golgi biogenesis. May regulate axonal development.<ref>PMID:14506257</ref> <ref>PMID:17321752</ref> <ref>PMID:18270207</ref> <ref>PMID:19665976</ref>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Atlastin, a member of the dynamin superfamily, is known to catalyse homotypic membrane fusion in the smooth endoplasmic reticulum (ER). Recent studies of atlastin have elucidated key features about its structure and function; however, several mechanistic details, including the catalytic mechanism and GTP hydrolysis-driven conformational changes, are yet to be determined. Here, we present the crystal structures of atlastin-1 bound to GDP.AlF(4)(-) and GppNHp, uncovering an intramolecular arginine finger that stimulates GTP hydrolysis when correctly oriented through rearrangements within the G domain. Utilizing Forster Resonance Energy Transfer, we describe nucleotide binding and hydrolysis-driven conformational changes in atlastin and their sequence. Furthermore, we discovered a nucleotide exchange mechanism that is intrinsic to atlastin's N-terminal domains. Our results indicate that the cytoplasmic domain of atlastin acts as a tether and homotypic interactions are timed by GTP binding and hydrolysis. Perturbation of these mechanisms may be implicated in a group of atlastin-associated hereditary neurodegenerative diseases.
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{{STRUCTURE_4idq| PDB=4idq | SCENE= }}
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Structural basis for conformational switching and GTP loading of the large G protein atlastin.,Byrnes LJ, Singh A, Szeto K, Benvin NM, O'Donnell JP, Zipfel WR, Sondermann H EMBO J. 2013 Jan 18. doi: 10.1038/emboj.2012.353. PMID:23334294<ref>PMID:23334294</ref>
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===human atlastin-1 1-446, N440T, GDPAlF4-===
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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{{ABSTRACT_PUBMED_23334294}}
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==See Also==
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*[[Atlastin|Atlastin]]
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==About this Structure==
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== References ==
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[[4idq]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4IDQ OCA].
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Benvin, N M.]]
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[[Category: Benvin, N M]]
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[[Category: Byrnes, L J.]]
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[[Category: Byrnes, L J]]
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[[Category: Donnell, J P.O.]]
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[[Category: Donnell, J P.O]]
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[[Category: Singh, A.]]
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[[Category: Singh, A]]
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[[Category: Sondermann, H.]]
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[[Category: Sondermann, H]]
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[[Category: Szeto, K.]]
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[[Category: Szeto, K]]
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[[Category: Zipfel, W R.]]
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[[Category: Zipfel, W R]]
[[Category: Gtp/gdp binding]]
[[Category: Gtp/gdp binding]]
[[Category: Gtpase]]
[[Category: Gtpase]]
[[Category: Hydrolase]]
[[Category: Hydrolase]]

Revision as of 16:51, 10 December 2014

human atlastin-1 1-446, N440T, GDPAlF4-

4idq, resolution 2.29Å

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