Orthogonal Strategies: Immunohistochemistry-Paraffin: FLNC Antibody [NBP1-89300] - Analysis in human skeletal muscle and liver tissues. Corresponding FLNC RNA-seq data are presented for the same tissues.
Immunocytochemistry/ Immunofluorescence: FLNC Antibody [NBP1-89300] - Staining of human cell line U-2 OS shows localization to plasma membrane and cytosol. Antibody staining shown in green.
Immunohistochemistry-Paraffin: FLNC Antibody [NBP1-89300] - Staining of human liver shows no positivity in hepatocytes as expected.
Immunohistochemistry-Paraffin: FLNC Antibody [NBP1-89300] - Staining of human skeletal muscle shows high expression.
Immunohistochemistry-Frozen: FLNC Antibody [NBP1-89300] - Mouse muscle vasus transverse cryosection stained with Filamin C antibody (NBP1-89300). IHC-Fr image submitted by a verified customer review.
Immunohistochemistry-Paraffin: FLNC Antibody [NBP1-89300] - Staning of human tonsil shows no positivity in non-germinal center cells as expected.
Immunohistochemistry-Paraffin: FLNC Antibody [NBP1-89300] - Staining of human prostate shows moderate cytoplasmic positivity in smooth muscle cells.
Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
PBS (pH 7.2) and 40% Glycerol
0.02% Sodium Azide
Immunogen affinity purified
Alternate Names for FLNC Antibody
ABPLABP-L, gamma filamin
filamin C, gamma (actin binding protein 280)
filamin C, gamma
FLN2actin binding protein 280
FLNC, Filamin-c, Filamin-2 (FLN2), Actin binding-like protein (APBL) (290.8 kDa) is an isoform of the Filamin proteins family. Filamins are broadly expressed and function as actin-binding and actin-crosslinking proteins. Structurally, Filamins consist of three main domains; N-terminal actin-binding domain, multiple central immunoglobulin (Ig) domains, and a C-terminal domain which supports homodimerization (1, 2). Functionally, Filamins play a role in cytoskeleton structure through interactions with actin, but they are implicated in a wide range of processes through their interactions with diverse molecular partners such as signaling molecules (MKK4, JNK1), ion channels (Kv4.2, Kir2.1), receptors (Insulin, Calcitonin), and transcription factors (Smad1-6, Foxc1) (3).
Three isoforms of Filamin are expressed in mammals, identified as FLNA, FLNB and FLNC. FLNC is considered a muscle specific Filamin isoform for its high expression in cardiac and skeletal muscles. In muscle tissue, FLNC localizes to the Z-line, sarcolemma, myotendinous and intercalated disks (2). FLNC interacts with various muscle proteins via a non-conserved sequence within its Ig domain 20. Some muscle binding partners for FLNC include gamma and delta-sarcoglycans in the sarcolemma, and FATZ, myozenins, myotilin and myopodin in the Z-discs (2, 4). FLNC variants are associated with various inherited pathological conditions including myofibrillar myopathy 5, distal myopathy 4, dilated cardiomyopathy, hypertrophic cardiomyopathy, and restrictive cardiomyopathy (2, 5).
1. Chiang, W., Greaser, M. L., & Lyons, G. E. (2000). Filamin isogene expression during mouse myogenesis. Developmental Dynamics. https://doi.org/10.1002/(sici)1097-0177(200001)217:1<99::aid-dvdy9>3.3.co;2-x
2. Leber, Y., Ruparelia, A. A., Kirfel, G., van der Ven, P. F. M., Hoffmann, B., Merkel, R., ... Furst, D. O. (2016). Filamin C is a highly dynamic protein associated with fast repair of myofibrillar microdamage. Human Molecular Genetics. https://doi.org/10.1093/hmg/ddw135
3. Nakamura, F., Stossel, T. P., & Hartwig, J. H. (2011). The filamins: Organizers of cell structure and function. Cell Adhesion and Migration. https://doi.org/10.4161/cam.5.2.14401
4. Thompson, T. G., Chan, Y. M., Hack, A. A., Brosius, M., Rajala, M., Lidov, H. G. W., ... Kunkel, L. M. (2000). Filamin 2 (FLN2): A muscle-specific sarcoglycan interacting protein. Journal of Cell Biology. https://doi.org/10.1083/jcb.148.1.115
5. Brodehl, A., Ferrier, R. A., Hamilton, S. J., Greenway, S. C., Brundler, M. A., Yu, W., ... Scherer, S. (2016). Mutations in FLNC are Associated with Familial Restrictive Cardiomyopathy. Human Mutation. https://doi.org/10.1002/humu.22942
This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.
Pathak, P., Blech-Hermoni, Y., Subedi, K. et al. Myopathy associated LDB3 mutation causes Z-disc disassembly and protein aggregation through PKC alpha and TSC2-mTOR downregulation. Commun Biol 4, 355 (2021). https://doi.org/10.1038/s42003-021-01864-1 ***Novus Innovators Program - new species or application used on a primary antibody.
Product General Protocols
Find general support by application which include: protocols, troubleshooting, illustrated assays, videos and webinars.
The concentration calculator allows you to quickly calculate the volume, mass or concentration of your vial. Simply enter your mass, volume, or concentration values for your reagent and the calculator will determine the rest.