FLNC Antibody [Alexa Fluor® 594]

Alexa Fluor (R) products are provided under an intellectual property license from Life Technologies Corporation. The purchase of this product conveys to the buyer the non-transferable right to use the purchased product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). The sale of this product is expressly conditioned on the buyer not using the product or its components, or any materials made using the product or its components, in any activity to generate revenue, which may include, but is not limited to use of the product or its components: (i) in manufacturing; (ii) to provide a service, information, or data in return for payment; (iii) for therapeutic, diagnostic or prophylactic purposes; or (iv) for resale, regardless of whether they are resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5791 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@lifetech.com. This conjugate is made on demand. Actual recovery may vary from the stated volume of this product. The volume will be greater than or equal to the unit size stated on the datasheet.

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).

References

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.

Array NBP2-79816AF594

• FLNC Antibodies
• FLNC ELISA Kits
• FLNC Proteins