Target intelligence / Profile preview

Filamin B (FLNB)

Target
FLNB
Molecular classification
Actin-binding protein, Cytoskeletal protein, Scaffolding protein, RNA-binding protein, Other
01

Overview

Filamin B (FLNB) is a large cytoskeletal actin-binding protein encoded by the FLNB gene on chromosome 3p14.3. It organizes actin filaments into orthogonal networks, anchoring them to cellular membranes and participating in scaffolding for transmembrane receptors. FLNB is essential for intracellular signaling, mechanotransduction, and the regulation of cell structure, shape, and motility, being particularly important in chondrocyte biology and bone development; mutations are a major cause of several congenital skeletal malformation syndromes. Beyond structural roles, FLNB also regulates transcription and alternative splicing, and its dysregulation has been linked to altered cell death, proliferation, and metastasis in cancer. Currently, FLNB is not a direct target of any approved drugs but is a relevant biomarker in clinical genetics for specific skeletal dysplasias.

Other names
Filamin-BFLN1LFLN3TABPTAPFLN-BFh1Truncated ABPABP-278FH1ABP-280 homologActin-binding-like proteinBeta-filaminFilamin homolog 1Filamin-3Thyroid autoantigenTruncated actin-binding proteinLarsen syndrome 1LRS1SCTAOI
02

Mechanism of action

Not applicable, as there are currently no drugs known to target FLNB directly.

03

Biological functions

Cytoskeleton organizationActin filament crosslinkingCell shape regulationCell motilityMechanotransductionSignal transductionRegulation of apoptosischondrocyte proliferation and differentiationTranscription and alternative splicing regulation
04

Disease associations

Skeletal dysplasia (including atelosteogenesis type I/III, boomerang dysplasia, Larsen syndrome, spondylocarpotarsal synostosis syndrome)Cancer (involved in tumorigenesis, metastasis, apoptosis regulation)Other developmental disorders
05

Safety considerations

Potential for deleterious or dominant negative phenotypes if targeted or suppressed (as FLNB mutations cause severe skeletal abnormalities)Essential in development; loss of function linked to lethal or severe developmental phenotypesNo data on drug-targeted safety concerns as no drugs are approved
06

Biomarkers

Mutations in FLNB are used as biomarkers in the genetic diagnosis of congenital skeletal disorders (e.g., atelosteogenesis type I/III, Larsen syndrome)

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