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| - | {{Seed}} | |
| - | [[Image:2d68.png|left|200px]] | |
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| - | <!--
| + | ==Structure of the N-terminal domain of FOP (FGFR1OP) protein== |
| - | The line below this paragraph, containing "STRUCTURE_2d68", creates the "Structure Box" on the page.
| + | <StructureSection load='2d68' size='340' side='right'caption='[[2d68]], [[Resolution|resolution]] 1.60Å' scene=''> |
| - | You may change the PDB parameter (which sets the PDB file loaded into the applet)
| + | == Structural highlights == |
| - | or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
| + | <table><tr><td colspan='2'>[[2d68]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2D68 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2D68 FirstGlance]. <br> |
| - | or leave the SCENE parameter empty for the default display.
| + | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.6Å</td></tr> |
| - | -->
| + | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2d68 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2d68 OCA], [https://pdbe.org/2d68 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2d68 RCSB], [https://www.ebi.ac.uk/pdbsum/2d68 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2d68 ProSAT]</span></td></tr> |
| - | {{STRUCTURE_2d68| PDB=2d68 | SCENE= }}
| + | </table> |
| - | | + | == Disease == |
| - | ===Structure of the N-terminal domain of FOP (FGFR1OP) protein===
| + | [https://www.uniprot.org/uniprot/CEP43_HUMAN CEP43_HUMAN] A chromosomal aberration involving CEP43 may be a cause of stem cell myeloproliferative disorder (MPD). Translocation t(6;8)(q27;p11) with FGFR1. MPD is characterized by myeloid hyperplasia, eosinophilia and T-cell or B-cell lymphoblastic lymphoma. In general it progresses to acute myeloid leukemia. The fusion proteins CEP43-FGFR1 or FGFR1-CEP43 may exhibit constitutive kinase activity and be responsible for the transforming activity (PubMed:9949182).<ref>PMID:9949182</ref> |
| - | | + | == Function == |
| - | | + | [https://www.uniprot.org/uniprot/CEP43_HUMAN CEP43_HUMAN] Required for anchoring microtubules to the centrosomes (PubMed:16314388, PubMed:28659385). Required for ciliation (PubMed:28625565, PubMed:28659385).<ref>PMID:16314388</ref> <ref>PMID:28625565</ref> <ref>PMID:28659385</ref> |
| - | <!-- | + | == References == |
| - | The line below this paragraph, {{ABSTRACT_PUBMED_16690081}}, adds the Publication Abstract to the page
| + | <references/> |
| - | (as it appears on PubMed at http://www.pubmed.gov), where 16690081 is the PubMed ID number.
| + | __TOC__ |
| - | -->
| + | </StructureSection> |
| - | {{ABSTRACT_PUBMED_16690081}}
| + | |
| - | | + | |
| - | ==About this Structure== | + | |
| - | 2D68 is a [[Single protein]] structure of 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=2D68 OCA].
| + | |
| - | | + | |
| - | ==Reference== | + | |
| - | Structure of the N-terminal domain of the FOP (FGFR1OP) protein and implications for its dimerization and centrosomal localization., Mikolajka A, Yan X, Popowicz GM, Smialowski P, Nigg EA, Holak TA, J Mol Biol. 2006 Jun 16;359(4):863-75. Epub 2006 Apr 24. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/16690081 16690081]
| + | |
| | [[Category: Homo sapiens]] | | [[Category: Homo sapiens]] |
| - | [[Category: Single protein]] | + | [[Category: Large Structures]] |
| - | [[Category: Mikolajka, A.]] | + | [[Category: Mikolajka A]] |
| - | [[Category: Alpha helical bundle]]
| + | |
| - | [[Category: Dimer]]
| + | |
| - | | + | |
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jul 28 17:36:13 2008''
| + | |
| Structural highlights
Disease
CEP43_HUMAN A chromosomal aberration involving CEP43 may be a cause of stem cell myeloproliferative disorder (MPD). Translocation t(6;8)(q27;p11) with FGFR1. MPD is characterized by myeloid hyperplasia, eosinophilia and T-cell or B-cell lymphoblastic lymphoma. In general it progresses to acute myeloid leukemia. The fusion proteins CEP43-FGFR1 or FGFR1-CEP43 may exhibit constitutive kinase activity and be responsible for the transforming activity (PubMed:9949182).[1]
Function
CEP43_HUMAN Required for anchoring microtubules to the centrosomes (PubMed:16314388, PubMed:28659385). Required for ciliation (PubMed:28625565, PubMed:28659385).[2] [3] [4]
References
- ↑ Popovici C, Zhang B, Grégoire MJ, Jonveaux P, Lafage-Pochitaloff M, Birnbaum D, Pébusque MJ. The t(6;8)(q27;p11) translocation in a stem cell myeloproliferative disorder fuses a novel gene, FOP, to fibroblast growth factor receptor 1. Blood. 1999 Feb 15;93(4):1381-9 PMID:9949182
- ↑ Yan X, Habedanck R, Nigg EA. A complex of two centrosomal proteins, CAP350 and FOP, cooperates with EB1 in microtubule anchoring. Mol Biol Cell. 2006 Feb;17(2):634-44. Epub 2005 Nov 28. PMID:16314388 doi:E05-08-0810
- ↑ Kanie T, Abbott KL, Mooney NA, Plowey ED, Demeter J, Jackson PK. The CEP19-RABL2 GTPase Complex Binds IFT-B to Initiate Intraflagellar Transport at the Ciliary Base. Dev Cell. 2017 Jul 10;42(1):22-36.e12. PMID:28625565 doi:10.1016/j.devcel.2017.05.016
- ↑ Mojarad BA, Gupta GD, Hasegan M, Goudiam O, Basto R, Gingras AC, Pelletier L. CEP19 cooperates with FOP and CEP350 to drive early steps in the ciliogenesis programme. Open Biol. 2017 Jun;7(6):170114. PMID:28659385 doi:10.1098/rsob.170114
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