3umn

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{{STRUCTURE_3umn| PDB=3umn | SCENE= }}
 
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===Crystal Structure of Lamin-B1===
 
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{{ABSTRACT_PUBMED_22265972}}
 
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==Disease==
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==Crystal Structure of Lamin-B1==
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[[http://www.uniprot.org/uniprot/LMNB1_HUMAN LMNB1_HUMAN]] Defects in LMNB1 are the cause of leukodystrophy demyelinating autosomal dominant adult-onset (ADLD) [MIM:[http://omim.org/entry/169500 169500]]. ADLD is a slowly progressive and fatal demyelinating leukodystrophy, presenting in the fourth or fifth decade of life. Clinically characterized by early autonomic abnormalities, pyramidal and cerebellar dysfunction, and symmetric demyelination of the CNS. It differs from multiple sclerosis and other demyelinating disorders in that neuropathology shows preservation of oligodendroglia in the presence of subtotal demyelination and lack of astrogliosis.<ref>PMID:16951681</ref>
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<StructureSection load='3umn' size='340' side='right'caption='[[3umn]], [[Resolution|resolution]] 2.00&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[3umn]] is a 3 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. This structure supersedes the now removed PDB entry [http://oca.weizmann.ac.il/oca-bin/send-pdb?obs=1&id=3hn9 3hn9]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3UMN OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3UMN FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/LMNB1_HUMAN LMNB1_HUMAN]] Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin.
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</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&#8491;</td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3umn FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3umn OCA], [https://pdbe.org/3umn PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3umn RCSB], [https://www.ebi.ac.uk/pdbsum/3umn PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3umn ProSAT]</span></td></tr>
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==About this Structure==
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</table>
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[[3umn]] is a 3 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. This structure supersedes the now removed PDB entry [http://oca.weizmann.ac.il/oca-bin/send-pdb?obs=1&id=3hn9 3hn9]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3UMN OCA].
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== Disease ==
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[https://www.uniprot.org/uniprot/LMNB1_HUMAN LMNB1_HUMAN] Defects in LMNB1 are the cause of leukodystrophy demyelinating autosomal dominant adult-onset (ADLD) [MIM:[https://omim.org/entry/169500 169500]. ADLD is a slowly progressive and fatal demyelinating leukodystrophy, presenting in the fourth or fifth decade of life. Clinically characterized by early autonomic abnormalities, pyramidal and cerebellar dysfunction, and symmetric demyelination of the CNS. It differs from multiple sclerosis and other demyelinating disorders in that neuropathology shows preservation of oligodendroglia in the presence of subtotal demyelination and lack of astrogliosis.<ref>PMID:16951681</ref>
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==Reference==
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== Function ==
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<ref group="xtra">PMID:022265972</ref><references group="xtra"/><references/>
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[https://www.uniprot.org/uniprot/LMNB1_HUMAN LMNB1_HUMAN] Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin.
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== References ==
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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: Amaya, M F.]]
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[[Category: Large Structures]]
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[[Category: Arrowsmith, C H.]]
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[[Category: Amaya MF]]
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[[Category: Bian, C B.]]
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[[Category: Arrowsmith CH]]
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[[Category: Bochkarev, A.]]
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[[Category: Bian CB]]
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[[Category: Bountra, C.]]
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[[Category: Bochkarev A]]
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[[Category: Edwards, A M.]]
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[[Category: Bountra C]]
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[[Category: Min, J.]]
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[[Category: Edwards AM]]
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[[Category: SGC, Structural Genomics Consortium.]]
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[[Category: Min J]]
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[[Category: Weigelt, J.]]
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[[Category: Weigelt J]]
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[[Category: Xu, C.]]
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[[Category: Xu C]]
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[[Category: Ig-like domain]]
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[[Category: Intermediate filament]]
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[[Category: Lipoprotein]]
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[[Category: Membrane]]
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[[Category: Nucleus]]
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[[Category: Sgc]]
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[[Category: Structural genomics consortium]]
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[[Category: Structural protein]]
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Current revision

Crystal Structure of Lamin-B1

PDB ID 3umn

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