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| [[Image:3hop.png|left|200px|thumb|Crystal structure of Human Filamin A complex with phosphate, [[3hop]]]]
| | <StructureSection load='2wa5' size='350' side='right' scene='' caption='Human Filamin B actin-binding domain complex with sulfate and carbonate (PDB code [[2wa5]]) '> |
| {{STRUCTURE_2wa5| PDB=2wa5 | SIZE=400| SCENE= |right|CAPTION=Human Filamin B actin-binding domain complex with sulfate and carbonate, [[2wa5]] }}
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| | __TOC__ |
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| | == Function == |
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| | * [[Filamin]] '''A (FLNA)''' has an actin-binding domain (ABD). It crosslinks actin filaments and participates in anchoring of membrane proteins<ref>PMID:19773341</ref>.<br /> |
| | * '''Filamin B (FLNB)''' is a human cytoplasmic protein which functions similarly to FLNA and guides proper skeletal development<ref>PMID:19505475</ref>.<br /> |
| | * '''Filamin C (FLNC)''' is functionally similar and contains 3 domains: the N-terminal ABD, the C-terminal dimerization domain (DD) and a membrane glycoprotein-binding domain<ref>PMID:11336782</ref>.<br /> |
| | For more details on filamin C see [[Group:MUZIC:FilaminC]]. |
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| | == Disease == |
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| | Mutations in FLNA cause frontometaphyseal dysplasia, intestinal pseudo-obstruction, Melnick-Needles syndrome, otopalatodigital syndrome and periventricular heterotopia. Mutations in FLNAB cause boomerang dysplasia<ref>PMID:25614868</ref>. |
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| | ==3D structures of filamin== |
| | [[Filamin 3D structures]] |
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| | | </StructureSection> |
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| [[Filamin]] '''A (FLNA)''' has an actin-binding domain (ABD). It crosslinks actin filaments and participates in anchoring of membrane proteins. '''Filamin B (FLNB)''' is a human cytoplasmic protein which functions similarly to FLNA and guides proper skeletal development. '''Filamin C (FLNC)''' is functionally similar and contains 3 domains: the N-terminal ABD, the C-terminal dimerization domain (DD) and a membrane glycoprotein-binding domain. For more details on filaminC see [[Group:MUZIC:FilaminC]]. The images at the left and at the right correspond to one representative filamin structure, ''i.e.'' the crystal structure of human filamin A ([[3hop]]), it forms a <scene name='Filamin/Cv/2'>dimer</scene> <ref>PMID:19773341</ref>. Two phosphate ions are rendered as space filling objects.
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| {{TOC limit|limit=2}}
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| ===3D structures of filamin===
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| Updated on {{REVISIONDAY2}}-{{MONTHNAME|{{REVISIONMONTH}}}}-{{REVISIONYEAR}}
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| == Filamin A ==
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| [[3isw]] – hFLNA repeat 21+CFTR peptide – human<br />
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| [[3rgh]] - hFLNA repeat 10<br />
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| [[2wfn]], [[3hop]], [[3hor]] – hFLNA ABD<br />
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| [[3hoc]] – hFLNA ABD (mutant)<br />
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| [[4m9p]] - hFLNA domains 3-5<br />
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| [[2aav]] - hFLNA domain 17 – NMR<br />
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| [[2bp3]] - hFLNA domain 17+GPIB peptide<br />
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| [[2k7p]] – hFLNA domains 16-17<br />
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| [[2k7q]] - hFLNA domains 18-19<br />
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| [[2j3s]] - hFLNA domains 19-21<br />
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| [[2w0p]] - hFLNA domain 21+migfilin peptide<br />
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| [[2jf1]], [[2brq]] - hFLNA domain 21+integrin peptide<br />
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| [[2k3t]] - hFLNA domain 23 – NMR<br />
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| [[3cnk]] - hFLNA DD<br />
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| == Filamin B ==
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| [[2wa5]], [[3fer]], [[2eea]] - hFLNB ABD<br />
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| [[2wa6]], [[2wa7]], [[4b7l]] - hFLNB ABD (mutant)<br />
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| [[2di9]], [[2dia]], [[2dib]], [[2dic]], [[2dj4]], [[2e9j]], [[2dmb]], [[2ee9]], [[2ee7]], [[2dmc]], [[2di8]], [[2dlg]], [[2e9i]], [[2ee6]], [[2eeb]], [[2eec]], [[2eed]] - hFLNB domains 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 20, 21, 22, 23, 24 – NMR<br />
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| == Filamin C ==
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| [[2k9u]] – hFLNC+migfilin peptide<br />
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| [[2d7m]], [[2d7n]], [[2d7o]], [[2d7p]] - hFLNC domains 14, 16, 17, 22 - NMR<br />
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| [[2nqc]] - hFLNC domain 21<br />
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| [[2d7q]] - hFLNC domain 23 – NMR<br />
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| [[3v8o]] - hFLNC domains 4,5 <br />
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| [[4mgx]] - hFLNC domains 4,5 + platelet glycoprotein peptide<br />
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| [[1v05]] - hFLNC DD<br />
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| == Other Filamins ==
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| [[1wlh]], [[1qfh]] – FLN rod domain – ''Dictyostelium discoideum''<br />
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| ==References== | | ==References== |
