Group:MUZIC:XIN
Xin actin-binding repeat-containing protein 1 (Alternative name: Cardiomyopathy-associated protein 1) is coded by the gene (Synonyms:CMYA1, XIN) synohas 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. the crystal structure of human filamin A (3hop), it forms a [1]. Two phosphate ions are rendered as space filling objects.
Filamin AFilamin A
3isw – hFLNA repeat 21+CFTR peptide – human
2wfn, 3hop, 3hor – hFLNA ABD
3hoc – hFLNA ABD (mutant)
2aav - hFLNA domain 17 – NMR
2bp3 - hFLNA domain 17+GPIB peptide
2k7p – hFLNA domains 16-17
2k7q - hFLNA domains 18-19
2j3s - hFLNA domains 19-21
2w0p - hFLNA domain 21+migfilin peptide
2jf1, 2brq - hFLNA domain 21+integrin peptide
2k3t - hFLNA domain 23 – NMR
3cnk - hFLNA DD
Filamin BFilamin B
2wa5, 3fer, 2eea - hFLNB ABD
2wa6, 2wa7 - hFLNB ABD (mutant)
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
Filamin CFilamin C
2k9u – hFLNC+migfilin peptide
2d7m, 2d7n, 2d7o, 2d7p - hFLNC domains 14, 16, 17, 22 - NMR
2nqc - hFLNC domain 21
2d7q - hFLNC domain 23 – NMR
1v05 - hFLNC DD
Other FilaminsOther Filamins
ReferencesReferences
- ↑ Clark AR, Sawyer GM, Robertson SP, Sutherland-Smith AJ. Skeletal dysplasias due to filamin A mutations result from a gain-of-function mechanism distinct from allelic neurological disorders. Hum Mol Genet. 2009 Dec 15;18(24):4791-800. Epub 2009 Sep 22. PMID:19773341 doi:10.1093/hmg/ddp442