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3jt0
From Proteopedia
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| - | + | ==Crystal Structure of the C-terminal fragment (426-558) Lamin-B1 from Homo sapiens, Northeast Structural Genomics Consortium Target HR5546A== | |
| - | + | <StructureSection load='3jt0' size='340' side='right' caption='[[3jt0]], [[Resolution|resolution]] 2.39Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | ==Disease== | + | <table><tr><td colspan='2'>[[3jt0]] is a 2 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=3JT0 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3JT0 FirstGlance]. <br> |
| - | [[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> | + | </td></tr><tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr> |
| - | + | <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[3hn9|3hn9]]</td></tr> | |
| - | ==Function== | + | <tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">LMN2, LMNB, LMNB1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr> |
| + | <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=3jt0 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3jt0 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3jt0 RCSB], [http://www.ebi.ac.uk/pdbsum/3jt0 PDBsum]</span></td></tr> | ||
| + | </table> | ||
| + | == Disease == | ||
| + | [[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> | ||
| + | == Function == | ||
[[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. | [[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. | ||
| - | + | == Evolutionary Conservation == | |
| - | == | + | [[Image:Consurf_key_small.gif|200px|right]] |
| - | [[ | + | Check<jmol> |
| - | + | <jmolCheckbox> | |
| - | == | + | <scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/jt/3jt0_consurf.spt"</scriptWhenChecked> |
| - | <references | + | <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked> |
| + | <text>to colour the structure by Evolutionary Conservation</text> | ||
| + | </jmolCheckbox> | ||
| + | </jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/chain_selection.php?pdb_ID=2ata ConSurf]. | ||
| + | <div style="clear:both"></div> | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
| - | [[Category: Abashidze, M | + | [[Category: Abashidze, M]] |
| - | [[Category: Acton, T B | + | [[Category: Acton, T B]] |
| - | [[Category: Belote,R L | + | [[Category: Belote,R L]] |
| - | [[Category: Ciccosanti,C | + | [[Category: Ciccosanti,C]] |
| - | [[Category: Everett, J K | + | [[Category: Everett, J K]] |
| - | [[Category: Hunt,J F | + | [[Category: Hunt,J F]] |
| - | [[Category: Kuzin,A | + | [[Category: Kuzin,A]] |
| - | [[Category: Montelione, G T | + | [[Category: Montelione, G T]] |
| - | [[Category: | + | [[Category: Structural genomic]] |
| - | [[Category: Nair, R | + | [[Category: Nair, R]] |
| - | [[Category: Rost, B | + | [[Category: Rost, B]] |
| - | [[Category: Sahdev,S | + | [[Category: Sahdev,S]] |
| - | [[Category: Seetharaman, J | + | [[Category: Seetharaman, J]] |
| - | [[Category: Tong,L | + | [[Category: Tong,L]] |
| - | [[Category: Xiao,R | + | [[Category: Xiao,R]] |
[[Category: Hr5546a]] | [[Category: Hr5546a]] | ||
[[Category: Intermediate filament]] | [[Category: Intermediate filament]] | ||
| Line 37: | Line 50: | ||
[[Category: Membrane]] | [[Category: Membrane]] | ||
[[Category: Nesg]] | [[Category: Nesg]] | ||
| - | [[Category: Northeast structural genomics consortium]] | ||
[[Category: Nucleus]] | [[Category: Nucleus]] | ||
[[Category: Phosphoprotein]] | [[Category: Phosphoprotein]] | ||
[[Category: Prenylation]] | [[Category: Prenylation]] | ||
| - | [[Category: Protein structure initiative | + | [[Category: PSI, Protein structure initiative]] |
| - | + | ||
| - | + | ||
[[Category: Structural protein]] | [[Category: Structural protein]] | ||
Revision as of 17:05, 18 December 2014
Crystal Structure of the C-terminal fragment (426-558) Lamin-B1 from Homo sapiens, Northeast Structural Genomics Consortium Target HR5546A
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Categories: Homo sapiens | Abashidze, M | Acton, T B | Belote,R L | Ciccosanti,C | Everett, J K | Hunt,J F | Kuzin,A | Montelione, G T | Structural genomic | Nair, R | Rost, B | Sahdev,S | Seetharaman, J | Tong,L | Xiao,R | Hr5546a | Intermediate filament | Lamin-b1 | Leukodystrophy | Lipoprotein | Lmnb1 human | Membrane | Nesg | Nucleus | Phosphoprotein | Prenylation | PSI, Protein structure initiative | Structural protein
