Gene Symbol: Flna
Description: filamin A
Alias: RGD1560614, filamin-A, Endothelial actin-binding protein 280), actin binding protein 280, filamin A, alpha, filamin, alpha
Species: rat
Products:     Flna

Top Publications

  1. Gorlin J, Yamin R, Egan S, Stewart M, Stossel T, Kwiatkowski D, et al. Human endothelial actin-binding protein (ABP-280, nonmuscle filamin): a molecular leaf spring. J Cell Biol. 1990;111:1089-105 pubmed
    ..The large size of the leaves, their interruption by two hinges and flexible actin-binding site, facilitate cross-linking of widely dispersed actin filaments. ..
  2. Ohta Y, Stossel T, Hartwig J. Ligand-sensitive binding of actin-binding protein to immunoglobulin G Fc receptor I (Fc gamma RI). Cell. 1991;67:275-82 pubmed
    ..This finding suggests the existence of a pathway communicating a signal between a functional IgG receptor and intracellular components involved in the effector responses to Fc gamma RI-ligand interaction. ..
  3. Feng Y, Chen M, Moskowitz I, Mendonza A, Vidali L, Nakamura F, et al. Filamin A (FLNA) is required for cell-cell contact in vascular development and cardiac morphogenesis. Proc Natl Acad Sci U S A. 2006;103:19836-41 pubmed
    Mutations in the human Filamin A (FLNA) gene disrupt neuronal migration to the cerebral cortex and cause cardiovascular defects...
  4. Kyndt F, Gueffet J, Probst V, Jaafar P, Legendre A, Le Bouffant F, et al. Mutations in the gene encoding filamin A as a cause for familial cardiac valvular dystrophy. Circulation. 2007;115:40-9 pubmed
    ..5-Mb region. A standard positional cloning approach identified a P637Q mutation in the filamin A (FLNA) gene in all affected members...
  5. Robertson S, Twigg S, Sutherland Smith A, Biancalana V, Gorlin R, Horn D, et al. Localized mutations in the gene encoding the cytoskeletal protein filamin A cause diverse malformations in humans. Nat Genet. 2003;33:487-91 pubmed
    Remodeling of the cytoskeleton is central to the modulation of cell shape and migration. Filamin A, encoded by the gene FLNA, is a widely expressed protein that regulates re-organization of the actin cytoskeleton by interacting with ..
  6. Wang H, Frankfurt M, Burns L. High-affinity naloxone binding to filamin a prevents mu opioid receptor-Gs coupling underlying opioid tolerance and dependence. PLoS ONE. 2008;3:e1554 pubmed publisher
    ..affinity than for the mu opioid receptor, naloxone binds a pentapeptide segment of the scaffolding protein filamin A, known to interact with the mu opioid receptor, to disrupt its chronic opioid-induced Gs coupling...
  7. Fox J. Identification of actin-binding protein as the protein linking the membrane skeleton to glycoproteins on platelet plasma membranes. J Biol Chem. 1985;260:11970-7 pubmed
  8. Fox J, Lamperti E, Eksioglu Y, Hong S, Feng Y, Graham D, et al. Mutations in filamin 1 prevent migration of cerebral cortical neurons in human periventricular heterotopia. Neuron. 1998;21:1315-25 pubmed
    ..FLN1 shows previously unrecognized, high-level expression in the developing cortex, is required for neuronal migration to the cortex, and is essential for embryogenesis. ..
  9. Zhang L, Bartley C, Gong X, Hsieh L, Lin T, Feliciano D, et al. MEK-ERK1/2-dependent FLNA overexpression promotes abnormal dendritic patterning in tuberous sclerosis independent of mTOR. Neuron. 2014;84:78-91 pubmed publisher
    ..Here, we found that the actin-crosslinking protein filamin A (FLNA) is overexpressed in tuberous sclerosis complex (TSC) mice, a PI3K-mTOR model of neurodevelopmental ..

More Information


  1. Sayner S, Balczon R, Frank D, Cooper D, Stevens T. Filamin A is a phosphorylation target of membrane but not cytosolic adenylyl cyclase activity. Am J Physiol Lung Cell Mol Physiol. 2011;301:L117-24 pubmed publisher
    ..The actin binding protein, filamin A, was detected in all membrane fractions...
  2. Liu G, Thomas L, Warren R, Enns C, Cunningham C, Hartwig J, et al. Cytoskeletal protein ABP-280 directs the intracellular trafficking of furin and modulates proprotein processing in the endocytic pathway. J Cell Biol. 1997;139:1719-33 pubmed
    ..The novel roles of ABP-280 and the cytoskeleton in the sorting of furin in the TGN/ endosomal system and the formation of proprotein processing compartments are discussed. ..
  3. Ghosh S, Ahrens W, Phatak S, Hwang S, Schrum L, Bonkovsky H. Association of filamin A and vimentin with hepatitis C virus proteins in infected human hepatocytes. J Viral Hepat. 2011;18:e568-77 pubmed publisher
    ..Using proteomics, qRT-PCR, Western blot and immunofluorescence techniques, we revealed that filamin A (fila) and vimentin (vim) were prominently increased proteins in HCV-expressing human hepatoma cells compared ..
  4. Abrass C, Hansen K. Insulin-like growth factor-binding protein-5-induced laminin gamma1 transcription requires filamin A. J Biol Chem. 2010;285:12925-34 pubmed publisher
    ..Similarly, filamin A (FLNa), an actin-binding protein that participates in cell attachment, plays important additional roles in ..
  5. Yamauchi T, Ishidao T, Nomura T, Shinagawa T, Tanaka Y, Yonemura S, et al. A B-Myb complex containing clathrin and filamin is required for mitotic spindle function. EMBO J. 2008;27:1852-62 pubmed publisher
    ..Thus, identification of the Myb-Clafi complex reveals a previously unrecognized function of B-Myb that may contribute to its role in chromosome stability, possibly, tumour suppression. ..
  6. Robertson S, Jenkins Z, Morgan T, Ades L, Aftimos S, Boute O, et al. Frontometaphyseal dysplasia: mutations in FLNA and phenotypic diversity. Am J Med Genet A. 2006;140:1726-36 pubmed
    ..leading to substitutions in the actin-binding domain and within filamin repeats 9, 10, 14, 16, 22, and 23 of filamin A in 13/23 (57%) of individuals in this cohort...
  7. Tirupula K, Ithychanda S, Mohan M, Naga Prasad S, Qin J, Karnik S. G protein-coupled receptors directly bind filamin A with high affinity and promote filamin phosphorylation. Biochemistry. 2015;54:6673-83 pubmed publisher
    Although interaction of a few G protein-coupled receptors (GPCRs) with Filamin A, a key actin cross-linking and biomechanical signal transducer protein, has been observed, a comprehensive structure-function analysis of this interaction ..
  8. Ramakrishnan N, Drescher M, Khan K, Hatfield J, Drescher D. HCN1 and HCN2 proteins are expressed in cochlear hair cells: HCN1 can form a ternary complex with protocadherin 15 CD3 and F-actin-binding filamin A or can interact with HCN2. J Biol Chem. 2012;287:37628-46 pubmed publisher
    ..alternate interactions of full-length proteins; HCN1 can interact with protocadherin 15 CD3 and F-actin-binding filamin A forming a complex that does not include HCN2, or HCN1 can interact with HCN2 forming a complex without ..
  9. Mizuhashi K, Kanamoto T, Moriishi T, Muranishi Y, Miyazaki T, Terada K, et al. Filamin-interacting proteins, Cfm1 and Cfm2, are essential for the formation of cartilaginous skeletal elements. Hum Mol Genet. 2014;23:2953-67 pubmed publisher
    ..These results suggest that Cfms are essential partner molecules of Flnb in regulating differentiation and proliferation of chondryocytes and actin dynamics. ..
  10. Peverelli E, Giardino E, Treppiedi D, Vitali E, Cambiaghi V, Locatelli M, et al. Filamin A (FLNA) plays an essential role in somatostatin receptor 2 (SST2) signaling and stabilization after agonist stimulation in human and rat somatotroph tumor cells. Endocrinology. 2014;155:2932-41 pubmed publisher
    ..The aim of this study was to investigate the role of cytoskeleton protein filamin A (FLNA) in SST2 expression and signaling in somatotroph tumor cells...
  11. Pi M, Spurney R, Tu Q, Hinson T, Quarles L. Calcium-sensing receptor activation of rho involves filamin and rho-guanine nucleotide exchange factor. Endocrinology. 2002;143:3830-8 pubmed
    ..Our results suggest that the C terminus of CasR may interact with filamin to create a cytoskeletal scaffold necessary for the spatial organization of Galphaq, RhoGEF Lbc, and Rho signaling pathways upstream of SRE activation. ..
  12. Stefanova M, Meinecke P, Gal A, Bolz H. A novel 9 bp deletion in the filamin a gene causes an otopalatodigital-spectrum disorder with a variable, intermediate phenotype. Am J Med Genet A. 2005;132A:386-90 pubmed
    ..We identified a novel in-frame deletion in exon 29 of the Filamin A gene (c.4904_4912del, p.R1635_V1637del) encoding rod domain repeat 14 of the protein...
  13. Berry F, O Neill M, Coca Prados M, Walter M. FOXC1 transcriptional regulatory activity is impaired by PBX1 in a filamin A-mediated manner. Mol Cell Biol. 2005;25:1415-24 pubmed
    ..Here we demonstrate that FOXC1 interacts with the actin-binding protein filamin A (FLNA)...
  14. Noam Y, Ehrengruber M, Koh A, Feyen P, Manders E, Abbott G, et al. Filamin A promotes dynamin-dependent internalization of hyperpolarization-activated cyclic nucleotide-gated type 1 (HCN1) channels and restricts Ih in hippocampal neurons. J Biol Chem. 2014;289:5889-903 pubmed publisher
    The actin-binding protein filamin A (FLNa) regulates neuronal migration during development, yet its roles in the mature brain remain largely obscure...
  15. Carabalona A, Beguin S, Pallesi Pocachard E, Buhler E, Pellegrino C, Arnaud K, et al. A glial origin for periventricular nodular heterotopia caused by impaired expression of Filamin-A. Hum Mol Genet. 2012;21:1004-17 pubmed publisher
    ..Mutations in the Filamin-A (or FLNA) gene are the main cause of PH, but the underlying pathological mechanism remains unknown...
  16. Gay O, Gilquin B, Nakamura F, Jenkins Z, McCartney R, Krakow D, et al. RefilinB (FAM101B) targets filamin A to organize perinuclear actin networks and regulates nuclear shape. Proc Natl Acad Sci U S A. 2011;108:11464-9 pubmed publisher
    ..We next identify the actin-binding filamin A (FLNA) protein as the downstream effector of refilins...
  17. Seebahn A, Rose M, Enz R. RanBPM is expressed in synaptic layers of the mammalian retina and binds to metabotropic glutamate receptors. FEBS Lett. 2008;582:2453-7 pubmed publisher
    ..RanBPM interacts with mGlu2 and other group II and group III receptors, except mGlu6. Our data suggest that RanBPM might be associated with mGlu receptors at synaptic sites. ..
  18. Kim H, Sengupta A, Glogauer M, McCulloch C. Filamin A regulates cell spreading and survival via beta1 integrins. Exp Cell Res. 2008;314:834-46 pubmed publisher
    ..We examined whether the actin-binding proteins cortactin, vinculin and filamin A are involved in the formation of the earliest extensions of cells spreading over collagen or poly-L-lysine-..
  19. Kainulainen T, Pender A, D Addario M, Feng Y, Lekic P, McCulloch C. Cell death and mechanoprotection by filamin a in connective tissues after challenge by applied tensile forces. J Biol Chem. 2002;277:21998-2009 pubmed
    ..There was a rapid (<10-s) decrease in plasma membrane potential after force application, which, in filamin A-deficient melanoma cells, contributed to irreversible cell depolarization...
  20. Bouvard D, Pouwels J, De Franceschi N, Ivaska J. Integrin inactivators: balancing cellular functions in vitro and in vivo. Nat Rev Mol Cell Biol. 2013;14:430-42 pubmed publisher
  21. Lesourne R, Fridman W, Daëron M. Dynamic interactions of Fc gamma receptor IIB with filamin-bound SHIP1 amplify filamentous actin-dependent negative regulation of Fc epsilon receptor I signaling. J Immunol. 2005;174:1365-73 pubmed
  22. Cho E, Cho D, Park J, Kurose H, Caron M, Kim K. Roles of protein kinase C and actin-binding protein 280 in the regulation of intracellular trafficking of dopamine D3 receptor. Mol Endocrinol. 2007;21:2242-54 pubmed
    ..A continuous interaction with the actin-binding protein 280 (filamin A), which was previously known to interact with D(3)R, is required for PMA-induced D(3)R sequestration...
  23. Ohta Y, Suzuki N, Nakamura S, Hartwig J, Stossel T. The small GTPase RalA targets filamin to induce filopodia. Proc Natl Acad Sci U S A. 1999;96:2122-8 pubmed
    ..RalA therefore is a downstream intermediate in Cdc42-mediated filopod production and uses filamin in this pathway. ..
  24. Hazrati L, Kleinschmidt DeMasters B, Handler M, Smith M, Ochi A, Otsubo H, et al. Astrocytic inclusions in epilepsy: expanding the spectrum of filaminopathies. J Neuropathol Exp Neurol. 2008;67:669-76 pubmed publisher
    ..spared distal subpial, or perivascular astrocytic processes and were strongly and exclusively immunopositive for filamin A, an actin binding protein involved in neuronal migration...
  25. Nagano T, Morikubo S, Sato M. Filamin A and FILIP (Filamin A-Interacting Protein) regulate cell polarity and motility in neocortical subventricular and intermediate zones during radial migration. J Neurosci. 2004;24:9648-57 pubmed
    ..We show here that Filamin A, a well known actin-binding protein, determines the shape of neocortical neurons during radial migration in vivo...
  26. Falet H, Pollitt A, Begonja A, Weber S, Duerschmied D, Wagner D, et al. A novel interaction between FlnA and Syk regulates platelet ITAM-mediated receptor signaling and function. J Exp Med. 2010;207:1967-79 pubmed publisher
    b>Filamin A (FlnA) cross-links actin filaments and connects the Von Willebrand factor receptor GPIb-IX-V to the underlying cytoskeleton in platelets...
  27. Aalberts J, van Tintelen J, Oomen T, Bergman J, Halley D, Jongbloed J, et al. Screening of TGFBR1, TGFBR2, and FLNA in familial mitral valve prolapse. Am J Med Genet A. 2014;164A:113-9 pubmed publisher
    So far only mutations in the filamin A gene (FLNA) have been identified as causing familial mitral valve prolapse (MVP). Previous studies have linked dysregulation of the transforming growth factor beta (TGF-?) cytokine family to MVP...
  28. Torrado M, Senatorov V, Trivedi R, Fariss R, Tomarev S. Pdlim2, a novel PDZ-LIM domain protein, interacts with alpha-actinins and filamin A. Invest Ophthalmol Vis Sci. 2004;45:3955-63 pubmed
    ..Pdlim2 was also expressed in the lung. In rat corneal and lung extracts, alpha-actinin-1, alpha-actinin-4, filamin A, and myosin heavy polypeptide 9 were co-immunoprecipitated with Pdlim2...
  29. Deng W, Lopez Camacho C, Tang J, Mendoza Villanueva D, Maya Mendoza A, Jackson D, et al. Cytoskeletal protein filamin A is a nucleolar protein that suppresses ribosomal RNA gene transcription. Proc Natl Acad Sci U S A. 2012;109:1524-9 pubmed publisher
    b>Filamin A (FLNA) is an actin-binding protein with a well-established role in the cytoskeleton, where it determines cell shape and locomotion by cross-linking actin filaments...
  30. Oegema R, Hulst J, Theuns Valks S, van Unen L, Schot R, Mancini G, et al. Novel no-stop FLNA mutation causes multi-organ involvement in males. Am J Med Genet A. 2013;161A:2376-84 pubmed publisher
    Mutations in FLNA (Filamin A, OMIM 300017) cause X-linked periventricular nodular heterotopia (XL-PNH). XL-PNH-associated mutations are considered lethal in hemizygous males...
  31. Su W, Mruk D, Lie P, Lui W, Cheng C. Filamin A is a regulator of blood-testis barrier assembly during postnatal development in the rat testis. Endocrinology. 2012;153:5023-35 pubmed
    ..However, the regulation of BTB assembly remains unknown. Herein, filamin A, an actin filament cross-linker that is known to maintain and regulate cytoskeleton structure and function in ..
  32. Adams M, Simms R, Abdelhamed Z, Dawe H, Szymanska K, Logan C, et al. A meckelin-filamin A interaction mediates ciliogenesis. Hum Mol Genet. 2012;21:1272-86 pubmed publisher
    ..Here, we found that the cytoplasmic domain of meckelin directly interacts with the actin-binding protein filamin A, potentially at the apical cell surface associated with the basal body...
  33. Nakamura F, Kumeta K, Hida T, Isono T, Nakayama Y, Kuramata Matsuoka E, et al. Amino- and carboxyl-terminal domains of Filamin-A interact with CRMP1 to mediate Sema3A signalling. Nat Commun. 2014;5:5325 pubmed publisher
    ..CRMP1(Ser522Asp) weakens the F-actin gelation crosslinked by Filamin-A. Thus, phosphorylated CRMP1 may remove Filamin-A from the actin cytoskeleton to facilitate its remodelling. ..
  34. Beekman J, van der Poel C, van der Linden J, van den Berg D, van den Berghe P, van de Winkel J, et al. Filamin A stabilizes Fc gamma RI surface expression and prevents its lysosomal routing. J Immunol. 2008;180:3938-45 pubmed
    b>Filamin A, or actin-binding protein 280, is a ubiquitously expressed cytosolic protein that interacts with intracellular domains of multiple receptors to control their subcellular distribution, and signaling capacity...
  35. Noam Y, Phan L, McClelland S, Manders E, Ehrengruber M, Wadman W, et al. Distinct regional and subcellular localization of the actin-binding protein filamin A in the mature rat brain. J Comp Neurol. 2012;520:3013-34 pubmed publisher
    b>Filamin A (FLNa) is an actin-binding protein that regulates cell motility, adhesion, and elasticity by cross-linking filamentous actin. Additional roles of FLNa include regulation of protein trafficking and surface expression...
  36. Klaile E, Müller M, Kannicht C, Singer B, Lucka L. CEACAM1 functionally interacts with filamin A and exerts a dual role in the regulation of cell migration. J Cell Sci. 2005;118:5513-24 pubmed
    The carcinoembryonic antigen-related cell adhesion molecule CEACAM1 (CD66a) and the scaffolding protein filamin A have both been implicated in tumor cell migration...
  37. Li M, Bermak J, Wang Z, Zhou Q. Modulation of dopamine D(2) receptor signaling by actin-binding protein (ABP-280). Mol Pharmacol. 2000;57:446-52 pubmed
    ..Our results suggest a new molecular mechanism of modulating D(2) receptor signaling by cytoskeletal protein interaction. ..
  38. Rafizadeh S, Zhang Z, Woltz R, Kim H, Myers R, Lu L, et al. Functional interaction with filamin A and intracellular Ca2+ enhance the surface membrane expression of a small-conductance Ca2+-activated K+ (SK2) channel. Proc Natl Acad Sci U S A. 2014;111:9989-94 pubmed publisher
    ..Here, we demonstrate that filamin A, a cytoskeletal protein, augments the trafficking of SK2 channels in cardiac myocytes...
  39. Wang H, Burns L. Naloxone's pentapeptide binding site on filamin A blocks Mu opioid receptor-Gs coupling and CREB activation of acute morphine. PLoS ONE. 2009;4:e4282 pubmed publisher
    ..This protective effect is mediated via a high-affinity interaction of NLX to a pentapeptide region in c-terminal filamin A (FLNA), a scaffolding protein interacting with MOR...
  40. Parrini E, Mei D, Pisanti M, Catarzi S, Pucatti D, Bianchini C, et al. Familial periventricular nodular heterotopia, epilepsy and Melnick-Needles Syndrome caused by a single FLNA mutation with combined gain-of-function and loss-of-function effects. J Med Genet. 2015;52:405-12 pubmed publisher
    Loss-of-function mutations of the FLNA gene cause a neuronal migration disorder defined as X-linked periventricular nodular heterotopia (PNH); gain-of-function mutations are associated with a group of X-linked skeletal dysplasias designed ..
  41. Ezzell R, Kenney D, Egan S, Stossel T, Hartwig J. Localization of the domain of actin-binding protein that binds to membrane glycoprotein Ib and actin in human platelets. J Biol Chem. 1988;263:13303-9 pubmed
    ..Because of the extended bipolar conformation of the ABP molecule, the data suggest that the GPIb.actin-binding region is located remote from the self-association, or dimerization, site of the ABP subunit. ..
  42. Allam A, Alvarez S, Brown M, Reyes L. Ureaplasma parvum infection alters filamin A dynamics in host cells. BMC Infect Dis. 2011;11:101 pubmed publisher
    ..One protein that was perturbed by infection (filamin A) was used to further elucidate the mechanism of U...
  43. Sheen V, Dixon P, Fox J, Hong S, Kinton L, Sisodiya S, et al. Mutations in the X-linked filamin 1 gene cause periventricular nodular heterotopia in males as well as in females. Hum Mol Genet. 2001;10:1775-83 pubmed
    ..These results demonstrate the sensitivity and specificity of DNA testing for FLN1 mutations and have important functional implications for models of FLN1 protein function in neuronal migration. ..
  44. Tigges U, Koch B, Wissing J, Jockusch B, Ziegler W. The F-actin cross-linking and focal adhesion protein filamin A is a ligand and in vivo substrate for protein kinase C alpha. J Biol Chem. 2003;278:23561-9 pubmed
    b>Filamin A is an established structural component of cell-matrix adhesion sites. In addition, it serves as a scaffold for the subcellular targeting of different signaling molecules...
  45. Fennell N, Foulds N, Johnson D, Wilson L, Wyatt M, Robertson S, et al. Association of mutations in FLNA with craniosynostosis. Eur J Hum Genet. 2015;23:1684-8 pubmed publisher
    Mutations of FLNA, an X-linked gene that encodes the cytoskeletal protein filamin A, cause diverse and distinct phenotypes including periventricular nodular heterotopia and otopalatodigital spectrum disorders (OPDS)...