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3jt0
From Proteopedia
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| - | {{STRUCTURE_3jt0| PDB=3jt0 | SCENE= }} | ||
| - | ===Crystal Structure of the C-terminal fragment (426-558) Lamin-B1 from Homo sapiens, Northeast Structural Genomics Consortium Target HR5546A=== | ||
| - | ==Disease== | + | ==Crystal Structure of the C-terminal fragment (426-558) Lamin-B1 from Homo sapiens, Northeast Structural Genomics Consortium Target HR5546A== |
| - | [[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> | + | <StructureSection load='3jt0' size='340' side='right'caption='[[3jt0]], [[Resolution|resolution]] 2.39Å' scene=''> |
| - | + | == Structural highlights == | |
| - | ==Function== | + | <table><tr><td colspan='2'>[[3jt0]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. 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> |
| + | </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> | ||
| + | <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 HUMAN])</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://pdbe.org/3jt0 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=3jt0 RCSB], [http://www.ebi.ac.uk/pdbsum/3jt0 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=3jt0 ProSAT]</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> |
| - | [[Category: | + | <text>to colour the structure by Evolutionary Conservation</text> |
| - | [[Category: Abashidze, M | + | </jmolCheckbox> |
| - | [[Category: Acton, T B | + | </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/main_output.php?pdb_ID=3jt0 ConSurf]. |
| - | [[Category: Belote,R L | + | <div style="clear:both"></div> |
| - | [[Category: Ciccosanti,C | + | == References == |
| - | [[Category: Everett, J K | + | <references/> |
| - | [[Category: Hunt,J F | + | __TOC__ |
| - | [[Category: Kuzin,A | + | </StructureSection> |
| - | [[Category: Montelione, G T | + | [[Category: Human]] |
| - | [[Category: | + | [[Category: Large Structures]] |
| - | [[Category: Nair, R | + | [[Category: Abashidze, M]] |
| - | [[Category: Rost, B | + | [[Category: Acton, T B]] |
| - | [[Category: Sahdev,S | + | [[Category: Belote, R L]] |
| - | [[Category: Seetharaman, J | + | [[Category: Ciccosanti, C]] |
| - | [[Category: Tong,L | + | [[Category: Everett, J K]] |
| - | [[Category: Xiao,R | + | [[Category: Hunt, J F]] |
| + | [[Category: Kuzin, A]] | ||
| + | [[Category: Montelione, G T]] | ||
| + | [[Category: Structural genomic]] | ||
| + | [[Category: Nair, R]] | ||
| + | [[Category: Rost, B]] | ||
| + | [[Category: Sahdev, S]] | ||
| + | [[Category: Seetharaman, J]] | ||
| + | [[Category: Tong, L]] | ||
| + | [[Category: Xiao, R]] | ||
| + | [[Category: Acetylation]] | ||
| + | [[Category: Chromosomal rearrangement]] | ||
| + | [[Category: Coiled coil]] | ||
[[Category: Hr5546a]] | [[Category: Hr5546a]] | ||
[[Category: Intermediate filament]] | [[Category: Intermediate filament]] | ||
| Line 37: | Line 55: | ||
[[Category: Membrane]] | [[Category: Membrane]] | ||
[[Category: Nesg]] | [[Category: Nesg]] | ||
| - | [[Category: Northeast structural genomics consortium]] | ||
[[Category: Nucleus]] | [[Category: Nucleus]] | ||
[[Category: Phosphoprotein]] | [[Category: Phosphoprotein]] | ||
| + | [[Category: Polymorphism]] | ||
[[Category: Prenylation]] | [[Category: Prenylation]] | ||
| - | [[Category: Protein structure initiative | + | [[Category: PSI, Protein structure initiative]] |
| - | + | ||
| - | + | ||
[[Category: Structural protein]] | [[Category: Structural protein]] | ||
Current revision
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: Human | Large Structures | 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 | Acetylation | Chromosomal rearrangement | Coiled coil | Hr5546a | Intermediate filament | Lamin-b1 | Leukodystrophy | Lipoprotein | Lmnb1 human | Membrane | Nesg | Nucleus | Phosphoprotein | Polymorphism | Prenylation | PSI, Protein structure initiative | Structural protein
