4ngc
From Proteopedia
(Difference between revisions)
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== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[4ngc]] 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=4NGC OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4NGC FirstGlance]. <br> | <table><tr><td colspan='2'>[[4ngc]] 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=4NGC OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4NGC FirstGlance]. <br> | ||
| - | </td></tr><tr><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[4ngb|4ngb]], [[4ngd|4ngd]], [[4ngf|4ngf]], [[4ngg|4ngg]], [[4nh3|4nh3]], [[4nh5|4nh5]], [[4nh6|4nh6]], [[4nha|4nha]]</td></tr> | + | </td></tr><tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[4ngb|4ngb]], [[4ngd|4ngd]], [[4ngf|4ngf]], [[4ngg|4ngg]], [[4nh3|4nh3]], [[4nh5|4nh5]], [[4nh6|4nh6]], [[4nha|4nha]]</td></tr> |
| - | <tr><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">DICER, DICER1, HERNA, KIAA0928 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr> | + | <tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">DICER, DICER1, HERNA, KIAA0928 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr> |
| - | <tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4ngc FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ngc OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4ngc RCSB], [http://www.ebi.ac.uk/pdbsum/4ngc PDBsum]</span></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=4ngc FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ngc OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4ngc RCSB], [http://www.ebi.ac.uk/pdbsum/4ngc PDBsum]</span></td></tr> |
| - | <table> | + | </table> |
== Disease == | == Disease == | ||
[[http://www.uniprot.org/uniprot/DICER_HUMAN DICER_HUMAN]] Defects in DICER1 are a cause of pleuropulmonary blastoma (PPB) [MIM:[http://omim.org/entry/601200 601200]]. PPB is a rare pediatric tumor of the lung that arises during fetal lung development and is often part of an inherited cancer syndrome. PPBs contain both epithelial and mesenchymal cells. Early in tumorigenesis, cysts form in lung airspaces, and these cysts are lined with benign-appearing epithelium. Mesenchymal cells susceptible to malignant transformation reside within the cyst walls and form a dense 'cambium' layer beneath the epithelial lining. In a subset of patients, overgrowth of the mesenchymal cells produces a sarcoma, a transition that is associated with a poorer prognosis.<ref>PMID:21882293</ref> <ref>PMID:19556464</ref> Defects in DICER1 are the cause of goiter multinodular type 1 with or without Sertoli-Leydig cell tumors (MNG1) [MIM:[http://omim.org/entry/138800 138800]]. A common disorder characterized by nodular overgrowth of the thyroid gland. Some individuals may also develop Sertoli-Leydig cell tumors, usually of the ovary.<ref>PMID:21882293</ref> <ref>PMID:21205968</ref> Note=DICER1 mutations have been found in uterine cervix embryonal rhabdomyosarcoma, primitive neuroectodermal tumor, Wilms tumor, pulmonary sequestration and juvenile intestinal polyp (PubMed:21882293). Somatic missense mutations affecting the RNase IIIb domain of DICER1 are common in non-epithelial ovarian tumors. These mutations do not abolish DICER1 function but alter it in specific cell types, a novel mechanism through which perturbation of microRNA processing may be oncogenic (PubMed:22187960).<ref>PMID:21882293</ref> | [[http://www.uniprot.org/uniprot/DICER_HUMAN DICER_HUMAN]] Defects in DICER1 are a cause of pleuropulmonary blastoma (PPB) [MIM:[http://omim.org/entry/601200 601200]]. PPB is a rare pediatric tumor of the lung that arises during fetal lung development and is often part of an inherited cancer syndrome. PPBs contain both epithelial and mesenchymal cells. Early in tumorigenesis, cysts form in lung airspaces, and these cysts are lined with benign-appearing epithelium. Mesenchymal cells susceptible to malignant transformation reside within the cyst walls and form a dense 'cambium' layer beneath the epithelial lining. In a subset of patients, overgrowth of the mesenchymal cells produces a sarcoma, a transition that is associated with a poorer prognosis.<ref>PMID:21882293</ref> <ref>PMID:19556464</ref> Defects in DICER1 are the cause of goiter multinodular type 1 with or without Sertoli-Leydig cell tumors (MNG1) [MIM:[http://omim.org/entry/138800 138800]]. A common disorder characterized by nodular overgrowth of the thyroid gland. Some individuals may also develop Sertoli-Leydig cell tumors, usually of the ovary.<ref>PMID:21882293</ref> <ref>PMID:21205968</ref> Note=DICER1 mutations have been found in uterine cervix embryonal rhabdomyosarcoma, primitive neuroectodermal tumor, Wilms tumor, pulmonary sequestration and juvenile intestinal polyp (PubMed:21882293). Somatic missense mutations affecting the RNase IIIb domain of DICER1 are common in non-epithelial ovarian tumors. These mutations do not abolish DICER1 function but alter it in specific cell types, a novel mechanism through which perturbation of microRNA processing may be oncogenic (PubMed:22187960).<ref>PMID:21882293</ref> | ||
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A Phosphate-Binding Pocket within the Platform-PAZ-Connector Helix Cassette of Human Dicer.,Tian Y, Simanshu DK, Ma JB, Park JE, Heo I, Kim VN, Patel DJ Mol Cell. 2014 Feb 20;53(4):606-16. doi: 10.1016/j.molcel.2014.01.003. Epub 2014 , Jan 30. PMID:24486018<ref>PMID:24486018</ref> | A Phosphate-Binding Pocket within the Platform-PAZ-Connector Helix Cassette of Human Dicer.,Tian Y, Simanshu DK, Ma JB, Park JE, Heo I, Kim VN, Patel DJ Mol Cell. 2014 Feb 20;53(4):606-16. doi: 10.1016/j.molcel.2014.01.003. Epub 2014 , Jan 30. PMID:24486018<ref>PMID:24486018</ref> | ||
| - | From | + | From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> |
</div> | </div> | ||
== References == | == References == | ||
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</StructureSection> | </StructureSection> | ||
[[Category: Human]] | [[Category: Human]] | ||
| - | [[Category: Patel, D J | + | [[Category: Patel, D J]] |
| - | [[Category: Simanshu, D K | + | [[Category: Simanshu, D K]] |
| - | [[Category: Tian, Y | + | [[Category: Tian, Y]] |
[[Category: Connector helix]] | [[Category: Connector helix]] | ||
[[Category: Endoribonuclease]] | [[Category: Endoribonuclease]] | ||
Revision as of 08:48, 5 January 2015
Structure of human Dicer Platform-PAZ-Connector Helix cassette in complex with 12-mer siRNA having UA-3' ends (2.1 Angstrom resolution)
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