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This is the first identified mutation in the dimerization domain of FLNB. This loss-of-function frameshift mutation in FLNB causes autosomal-recessive SCT with rarely reported rib anomalies. This report demonstrates the involvement of rib anomaly in SCT and its causative mutation in the dimerization domain of FLNB.
Our results provide evidence for the involvement of FLNB in the pathogenesis of isolated Congenital talipes equinovarusand have expanded the clinical spectrum of FLNB mutations.
FlnA (show FLNA Proteins) more strongly binds RhoA (show RHOA Proteins), although both filamins overlap with RhoA (show RHOA Proteins) expression in the cell cytoplasm. FlnA (show FLNA Proteins) promotes RhoA (show RHOA Proteins) activation whereas FlnB indirectly inhibits this pathway. Moreover, FlnA (show FLNA Proteins) loss leads to diminished expression of b1-integrin, whereas FlnB loss promotes integrin expression
splicing variants of FLNB are differentially expressed in giant cell tumor cells and may play a role in the proliferation and differentiation of tumor cells.
F-actin clustering through the interaction with the mutant FLNB actin-binding domain may limit the cytoskeletal reorganization, preventing normal skeletal development.
FLNb enhances invasion of cancer cells through phosphorylation of MRLC and FAK (show PTK2 Proteins).
Polymorphism at rs11720285, rs11130605 and rs9809315, all of which are located either 5' of the transcription start site or in intron 1 of the FLNB gene has been identified as significantly associated with BMD (show BEST1 Proteins) in Caucasian women.
study presents two patients with Atelosteogenesis Type I caused by two novel Filamin B (FLNB) mutations affecting the same FLNB residue: c.542G > A, predicting p.Gly181Asp and c.542G > C, predicting p.Gly181Arg
VEGF (show VEGFA Proteins) and PKC (show PRRT2 Proteins) promote degradation-independent protein ubiquitination of FLNB to control intracellular trafficking of HDAC7 (show HDAC7 Proteins).
The structure reveals a new hinge in the linker region between actin binding domain (ABD) and the first filamin repeat that is ideally positioned to orient the ABD for actin binding.
These findings indicate that FLNB is involved in attenuation of TGFb/BMP signaling and influences annulus fibrosus cell fate
Fmn1 (show FMN1 Proteins) and FlnB have shared and independent functions.
Filamin a, b-interacting proteins, Cfm1 and Cfm2, are essential for the formation of cartilaginous skeleton.
FlnB loss reduced Cdk1 (show CDK1 Proteins) phosphorylation (an inhibitor of G2/M phase progression) and Cdk1 (show CDK1 Proteins) inhibition in chondrocytes mimicked the null FlnB, premature differentiation phenotype, through a beta1-integrin receptor- Pi3k/Akt (show AKT1 Proteins) mediated pathway.
these data demonstrate that coordinated expression of GPIbalpha (show GP1BA Proteins) and filamin (show FLNA Proteins) is required for efficient trafficking of either protein to the cell surface, and for production of normal-sized platelets.
Filamins A and B have a major role in the maintenance of actin-based mechanical linkages that enable endoplasmic spreading and microtubule extension as well as sustained traction forces and mature focal adhesions.
Filamin B deficiency in mice results in skeletal malformations and impaired microvascular development
disruption of the ECM (show MMRN1 Proteins)-beta1-integrin-Flnb pathway contributes to defects in vertebral and distal limb development, similar to those seen in the human autosomal recessive SCT (show SECR Proteins) due to Flnb mutations
Flnb represses chondrocyte hypertrophy in a Runx2 (show RUNX2 Proteins)/Smad3 (show SMAD3 Proteins)-dependent manner.
Disruption of the Flnb gene in mice phenocopies the human disease spondylocarpotarsal synotosis syndrome.
This gene encodes a member of the filamin family. The encoded protein interacts with glycoprotein Ib alpha as part of the process to repair vascular injuries. The platelet glycoprotein Ib complex includes glycoprotein Ib alpha, and it binds the actin cytoskeleton. Mutations in this gene have been found in several conditions: atelosteogenesis type 1 and type 3\; boomerang dysplasia\; autosomal dominant Larsen syndrome\; and spondylocarpotarsal synostosis syndrome. Multiple alternatively spliced transcript variants that encode different protein isoforms have been described for this gene.
filamin B, beta (actin binding protein 278)
, filamin B, beta
, ABP-280 homolog
, Larsen syndrome 1 (autosomal dominant)
, actin binding protein 278
, actin-binding-like protein
, filamin homolog 1
, thyroid autoantigen
, filamin, beta
, filamin B beta
, retina filamin
, ABP-280-like protein