Filamin: Difference between revisions

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[[Image:3hop.png|left|200px|thumb|Crystal structure of Human Filamin A, [[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_3hop|  PDB=3hop  | SIZE=300| SCENE=Filamin/Cv/1 |right|CAPTION=Human Filamin A, [[3hop]] }}
[[Filamins|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. The images at the left and at the right correspond to one representative Filamin structure, ''i.e.'' crystal structure of Human Filamin A ([[3hop]]), it forms a <scene name='Filamin/Cv/2'>dimer</scene> <ref>PMID:19773341</ref>.


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== Filamin A ==


== Function ==


[[3isw]] – hFLNA repeat 21+CFTR peptide – human<br />
* [[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 />
[[2wfn]], [[3hop]], [[3hor]] – hFLNA ABD<br />
* '''Filamin B (FLNB)''' is a human cytoplasmic protein which functions similarly to FLNA and guides proper skeletal development<ref>PMID:19505475</ref>.<br />
[[3hoc]] – hFLNA ABD (mutant)<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 />
[[2aav]] - hFLNA domain 17 – NMR<br />
For more details on filamin C see [[Group:MUZIC:FilaminC]].
[[2bp3]] - hFLNA domain 17+GPIB peptide<br />  
[[2k7p]] – hFLNA domains 16-17<br />
[[2k7q]] - hFLNA domains 18-19<br />
[[2j3s]] - hFLNA domains 19-21<br />
[[2w0p]] - hFLNA domain 21+migfilin peptide<br />
[[2jf1]], [[2brq]] - hFLNA domain 21+integrin peptide<br />
[[2k3t]] - hFLNA domain 23 – NMR<br />
[[3cnk]] - hFLNA DD<br />


== Disease ==


== Filamin B ==
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>.


==3D structures of filamin==
[[Filamin 3D structures]]


[[2wa5]], [[3fer]] - hFLNB  ABD<br />
</StructureSection>
[[2wa6]], [[2wa7]] - hFLNB  ABD (mutant)<br />
[[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 />
 
 
== Filamin C ==
 
 
[[2k9u]] – hFLNC+migfilin peptide<br />
[[2d7m]], [[2d7n]], [[2d7o]], [[2d7p]] - hFLNC domains 14, 16, 17, 22 - NMR<br />
[[2nqc]] - hFLNC domain 21<br />
[[2d7q]] - hFLNC domain 23 – NMR<br />
[[1v05]] - hFLNC DD<br />
 
 
== Other Filamins ==
 
 
[[1wlh]], [[1qfh]] – FLN rod domain – Dictyostelium discoideum<br />


==References==
==References==
<references />
<references />
[[Category:Topic Page]]

Latest revision as of 09:05, 1 July 2019

Human Filamin B actin-binding domain complex with sulfate and carbonate (PDB code 2wa5)

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References

Proteopedia Page Contributors and Editors (what is this?)

Alexander Berchansky, Jaime Prilusky, Michal Harel