Protein: P21333

Uniprot: Promotes orthogonal branching of actin filaments and links actin filaments to membrane glycoproteins. Anchors various transmembrane proteins to the actin cytoskeleton and serves as a scaffold for a wide range of cytoplasmic signaling proteins. Interaction with FLNA may allow neuroblast migration from the ventricular zone into the cortical plate. Tethers cell surface-localized furin, modulates its rate of internalization and directs its intracellular trafficking (By similarity). Involved in ciliogenesis. Plays a role in cell-cell contacts and adherens junctions during the development of blood vessels, heart and brain organs. Plays a role in platelets morphology through interaction with SYK that regulates ITAM- and ITAM-like-containing receptor signaling, resulting in by platelet cytoskeleton organization maintenance (By similarity). more..
GO ID 1 Function 1 Module ID 1 GO ID 2 Function 2 Module ID 2 Association Probability (PrOnto) Interaction Probability (PrOnto)
GO:0007166 cell surface receptor signaling pathway 108 GO:0034641 cellular nitrogen compound metabolic process 35 5.53e-55 9.57e-64
Module ID (MoonGO) GO ID (BP) GO Name
35 GO:0044271 cellular nitrogen compound biosynthetic process
35 GO:0044267 cellular protein metabolic process
35 GO:0090304 nucleic acid metabolic process
35 GO:0010468 regulation of gene expression
35 GO:0009966 regulation of signal transduction
108 GO:0007167 enzyme linked receptor protein signaling pathway
108 GO:0006139 nucleobase-containing compound metabolic process
108 GO:0031325 positive regulation of cellular metabolic process
108 GO:1902533 positive regulation of intracellular signal transduction
108 GO:0010604 positive regulation of macromolecule metabolic process
108 GO:0006468 protein phosphorylation
306 GO:0044267 cellular protein metabolic process
306 GO:0060255 regulation of macromolecule metabolic process
306 GO:0007165 signal transduction
479 GO:0016070 RNA metabolic process
479 GO:0034645 cellular macromolecule biosynthetic process
479 GO:0044271 cellular nitrogen compound biosynthetic process
479 GO:0044267 cellular protein metabolic process
479 GO:0010468 regulation of gene expression
479 GO:0007165 signal transduction
498 GO:0060255 regulation of macromolecule metabolic process
750 GO:0048468 cell development
750 GO:0060255 regulation of macromolecule metabolic process
750 GO:0007165 signal transduction
Module ID (MoonGO) GO ID (CC) GO Name
35 GO:0005829 cytosol
35 GO:0005634 nucleus
35 GO:0005886 plasma membrane
108 GO:0005829 cytosol
108 GO:0005634 nucleus
108 GO:0005886 plasma membrane
306 GO:0070062 extracellular exosome
479 GO:0070013 intracellular organelle lumen
479 GO:0044428 nuclear part
498 GO:0005634 nucleus
634 GO:0005829 cytosol
634 GO:0005634 nucleus
750 GO:0005829 cytosol
750 GO:0043232 intracellular non-membrane-bounded organelle
750 GO:0005634 nucleus
750 GO:0005886 plasma membrane
GO ID (BP) GO Name Evidence Code (GO EC)
GO:0002576 platelet degranulation TAS
GO:0007195 adenylate cyclase-inhibiting dopamine receptor signaling pathway IMP
GO:0016479 negative regulation of transcription from RNA polymerase I promoter IDA
GO:0021943 formation of radial glial scaffolds IEA
GO:0021987 cerebral cortex development IEA
GO:0030168 platelet activation TAS
GO:0030334 regulation of cell migration IDA
GO:0031532 actin cytoskeleton reorganization IDA
GO:0034329 cell junction assembly TAS
GO:0034394 protein localization to cell surface IDA
GO:0042177 negative regulation of protein catabolic process IMP
GO:0042789 mRNA transcription from RNA polymerase II promoter IEA
GO:0042993 positive regulation of transcription factor import into nucleus IMP
GO:0043066 negative regulation of apoptotic process IMP
GO:0043113 receptor clustering IDA
GO:0043123 positive regulation of I-kappaB kinase/NF-kappaB signaling IMP
GO:0043433 negative regulation of DNA binding transcription factor activity IDA
GO:0044319 wound healing, spreading of cells IDA
GO:0045184 establishment of protein localization IDA
GO:0050821 protein stabilization IMP
GO:0051220 cytoplasmic sequestering of protein IMP
GO:0051764 actin crosslink formation IDA
GO:0060271 cilium assembly IMP
GO:0070527 platelet aggregation IMP
GO:0071526 semaphorin-plexin signaling pathway IGI
GO:0072659 protein localization to plasma membrane IDA
GO:0090307 mitotic spindle assembly IDA
GO:1900026 positive regulation of substrate adhesion-dependent cell spreading IMP
GO:1901381 positive regulation of potassium ion transmembrane transport IDA
GO:1905000 regulation of membrane repolarization during atrial cardiac muscle cell action potential IC
GO:1905031 regulation of membrane repolarization during cardiac muscle cell action potential ISS
GO:2000179 positive regulation of neural precursor cell proliferation IEA
GO:2001046 positive regulation of integrin-mediated signaling pathway IMP
GO:2001224 positive regulation of neuron migration IEA
GO ID (CC) GO Name Evidence Code (GO EC)
GO:0005576 extracellular region TAS
GO:0005634 nucleus IDA
GO:0005730 nucleolus IMP
GO:0005737 cytoplasm IDA
GO:0005829 cytosol TAS
GO:0005886 plasma membrane IDA
GO:0005911 cell-cell junction IDA
GO:0005925 focal adhesion IDA
GO:0015629 actin cytoskeleton IC
GO:0016020 membrane IDA
GO:0030018 Z disc ISS
GO:0030863 cortical cytoskeleton IEA
GO:0031012 extracellular matrix IDA
GO:0031523 Myb complex IDA
GO:0031941 filamentous actin IDA
GO:0043025 neuronal cell body IEA
GO:0043198 dendritic shaft IEA
GO:0048471 perinuclear region of cytoplasm IEA
GO:0070062 extracellular exosome IDA
GO:0097440 apical dendrite IEA
GO ID 1 Component 1 GO ID 2 Component 2 Association Probability (PrOnto) Interaction Probability (PrOnto)
GO:0005576 extracellular region GO:0005634 nucleus 4.48e-91 1.28e-107
GO:0005576 extracellular region GO:0005730 nucleolus 6.04e-15 4.85e-05
GO:0005634 nucleus GO:0005886 plasma membrane 5.35e-300 7.52e-165
GO:0005634 nucleus GO:0016020 membrane 0.00e+00 4.39e-236
GO:0005634 nucleus GO:0031012 extracellular matrix 1.58e-13 4.18e-03
GO:0005634 nucleus GO:0070062 extracellular exosome 6.30e-13 4.54e-26
GO:0005730 nucleolus GO:0005886 plasma membrane 7.70e-47 3.09e-07
GO:0005730 nucleolus GO:0016020 membrane 8.89e-46 1.83e-10
GO:0005829 cytosol GO:0031012 extracellular matrix 4.46e-06 5.18e-08
PMID Article Title
2248958 Purification of human smooth muscle filamin and characterization of structural domains and functional sites.
2391361 Human endothelial actin-binding protein (ABP-280, nonmuscle filamin): a molecular leaf spring.
7689010 Mapping of two genes encoding isoforms of the actin binding protein ABP-280, a dystrophin like protein, to Xq28 and to chromosome 7.
8088819 The exon-intron organization of the human X-linked gene (FLN1) encoding actin-binding protein 280.
8733135 Long-range sequence analysis in Xq28: thirteen known and six candidate genes in 219.4 kb of high GC DNA between the RCP/GCP and G6PD loci.
9437013 Interaction of presenilins with the filamin family of actin-binding proteins.
11102480 Localization and enhanced current density of the Kv4.2 potassium channel by interaction with the actin-binding protein filamin.
11153914 Genomic structure and fine mapping of the two human filamin gene paralogues FLNB and FLNC and comparative analysis of the filamin gene family.
11252955 Filamins as integrators of cell mechanics and signalling.
11336782 Structural and functional aspects of filamins.
11532987 Mutations in the X-linked filamin 1 gene cause periventricular nodular heterotopia in males as well as in females.
11739414 The SH2-containing inositol polyphosphate 5-phosphatase, SHIP-2, binds filamin and regulates submembraneous actin.
11914408 Familial periventricular heterotopia: missense and distal truncating mutations of the FLN1 gene.
12393796 Filamin A and filamin B are co-expressed within neurons during periods of neuronal migration and can physically interact.
12410386 Bilateral periventricular nodular heterotopia due to filamin 1 gene mutation: widespread glomeruloid microvascular anomaly and dysplastic cytoarchitecture in the cerebral cortex.
12612583 Localized mutations in the gene encoding the cytoskeletal protein filamin A cause diverse malformations in humans.
14702039 Complete sequencing and characterization of 21,243 full-length human cDNAs.
15249610 Germline and mosaic mutations of FLN1 in men with periventricular heterotopia.
15654694 A novel 9 bp deletion in the filamin A gene causes an otopalatodigital-spectrum disorder with a variable, intermediate phenotype.
15668422 Filamin A mutations cause periventricular heterotopia with Ehlers-Danlos syndrome.
15684392 FOXC1 transcriptional regulatory activity is impaired by PBX1 in a filamin A-mediated manner.
15772651 The DNA sequence of the human X chromosome.
15940695 A novel filamin A D203Y mutation in a female patient with otopalatodigital type 1 syndrome and extremely skewed X chromosome inactivation.
15994863 Ehlers-Danlos syndrome and periventricular nodular heterotopia in a Spanish family with a single FLNA mutation.
16076904 The Z-disc proteins myotilin and FATZ-1 interact with each other and are connected to the sarcolemma via muscle-specific filamins.
16291724 CEACAM1 functionally interacts with filamin A and exerts a dual role in the regulation of cell migration.
16299064 A filamin A splice mutation resulting in a syndrome of facial dysmorphism, periventricular nodular heterotopia, and severe constipation reminiscent of cerebro-fronto-facial syndrome.
16596676 Genotype-epigenotype-phenotype correlations in females with frontometaphyseal dysplasia.
16862148 FilGAP, a Rho- and ROCK-regulated GAP for Rac binds filamin A to control actin remodelling.
16964243 A probability-based approach for high-throughput protein phosphorylation analysis and site localization.
17081983 Global, in vivo, and site-specific phosphorylation dynamics in signaling networks.
17190868 Mutations in the gene encoding filamin A as a cause for familial cardiac valvular dystrophy.
17357080 Filamin A is mutated in X-linked chronic idiopathic intestinal pseudo-obstruction with central nervous system involvement.
17431908 Otopalatodigital syndrome type 2 in two siblings with a novel filamin A 629G>T mutation: clinical, pathological, and molecular findings.
17632775 Filamin A mutation is one cause of FG syndrome.
17690686 Structure of three tandem filamin domains reveals auto-inhibition of ligand binding.
17924679 Improved titanium dioxide enrichment of phosphopeptides from HeLa cells and high confident phosphopeptide identification by cross-validation of MS/MS and MS/MS/MS spectra.
18088087 Phosphoproteome of resting human platelets.
18220336 Combining protein-based IMAC, peptide-based IMAC, and MudPIT for efficient phosphoproteomic analysis.
18322202 Filamin A stabilizes FcgammaRI surface expression and prevents its lysosomal routing.
18556573 ECSM2, an endothelial specific filamin a binding protein that mediates chemotaxis.
18669648 A quantitative atlas of mitotic phosphorylation.
18691976 Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle.
19367720 Phosphorylation analysis of primary human T lymphocytes using sequential IMAC and titanium oxide enrichment.
19413330 Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach.
19608861 Lysine acetylation targets protein complexes and co-regulates major cellular functions.
19622754 Atomic structures of two novel immunoglobulin-like domain pairs in the actin cross-linking protein filamin.
19690332 Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions.
19828450 Identification and characterization of multiple similar ligand-binding repeats in filamin: implication on filamin-mediated receptor clustering and cross-talk.
19923718 Structure of the human filamin A actin-binding domain.
20068231 Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis.
20598277 Terminal osseous dysplasia is caused by a single recurrent mutation in the FLNA gene.
20713593 A novel interaction between FlnA and Syk regulates platelet ITAM-mediated receptor signaling and function.
20736409 Systematic mapping and functional analysis of a family of human epididymal secretory sperm-located proteins.
21228480 Identification of novel nuclear protein interactions with the N-terminal part of filamin A.
21269460 Initial characterization of the human central proteome.
21406692 System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation.
21524097 Evidence for multisite ligand binding and stretching of filamin by integrin and migfilin.
21697133 Full-length transcriptome analysis of human retina-derived cell lines ARPE-19 and Y79 using the vector-capping method.
21960593 Thrombocytopenia resulting from mutations in filamin A can be expressed as an isolated syndrome.
22121117 A meckelin-filamin A interaction mediates ciliogenesis.
23037936 Congenital short bowel syndrome as the presenting symptom in male patients with FLNA mutations.
23186163 Toward a comprehensive characterization of a human cancer cell phosphoproteome.
23890175 Junctional Rab13-binding protein (JRAB) regulates cell spreading via filamins.
24052262 ASB2alpha, an E3 ubiquitin ligase specificity subunit, regulates cell spreading and triggers proteasomal degradation of filamins by targeting the filamin calponin homology 1 domain.
24275569 An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
25114211 Mapping of SUMO sites and analysis of SUMOylation changes induced by external stimuli.
25218447 Uncovering global SUMOylation signaling networks in a site-specific manner.
25666618 A mechanism of global shape-dependent recognition and phosphorylation of filamin by protein kinase A.
25755297 System-wide analysis of SUMOylation dynamics in response to replication stress reveals novel small ubiquitin-like modified target proteins and acceptor lysines relevant for genome stability.
25772364 SUMO-2 orchestrates chromatin modifiers in response to DNA damage.
25944712 N-terminome analysis of the human mitochondrial proteome.
26460884 G Protein-Coupled Receptors Directly Bind Filamin A with High Affinity and Promote Filamin Phosphorylation.
26766444 Endothelial RSPO3 controls vascular stability and pruning through non-canonical WNT/Ca(2+)/NFAT signaling.
27193221 Otopalatodigital spectrum disorders: refinement of the phenotypic and mutational spectrum.
28112733 Site-specific mapping of the human SUMO proteome reveals co-modification with phosphorylation.
OMIM ID Disease Name
300048 Congenital short bowel syndrome, X-linked
300048 Intestinal pseudoobstruction, neuronal, chronic idiopathic, X-linked
300049 Periventricular nodular heterotopia 1
300244 Terminal osseous dysplasia
300321 FG syndrome 2
Domain Name Domain ID Source
A37098 A37098 PIR
Filamin/ABP280_rpt IPR001298 InterPro
Actinin_actin-bd_CS IPR001589 InterPro
CH-domain IPR001715 InterPro
Ig-like_fold IPR013783 InterPro
Ig_E-set IPR014756 InterPro
Filamin/ABP280_repeat-like IPR017868 InterPro
FLN_A IPR028559 InterPro
Calponin-like_dom_sf IPR036872 InterPro
CH PF00307 Pfam
Filamin PF00630 Pfam
SSF47576 SSF47576 SUPFAM
SSF81296 SSF81296 SUPFAM
CH cd00014 CDD