FLNC Antibody

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.

• Reactivity: Hu, Mu, Rt
• Applications: WB, ICC/IF, IHC
• Clonality: Polyclonal
• Host: Rabbit
• Conjugate: Unconjugated

FLNC Antibody Summary

• Immunogen: This antibody was developed against Recombinant Protein corresponding to amino acids: HSLHETSTVLVETVTKSSSSRGSSYSSIPKFSSDASKVVTRGPGLSQAFVGQKNSFTVDCSKAGTNMMMVGVHGPKTPCEEVYVKHMGNRVYNVTYTVKEKGDY
• Predicted Species: Mouse (99%), Rat (99%). Backed by our 100% Guarantee.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Rabbit
• Gene: FLNC
• Purity: Immunogen affinity purified

Applications/Dilutions

• Dilutions:
  • Immunocytochemistry/Immunofluorescence 0.25 - 2 ug/mL
  • Immunohistochemistry 1:200 - 1:500
  • Immunohistochemistry-Frozen -Validated from a verified customer review.
  • Immunohistochemistry-Paraffin 1:200 - 1:500
  • Western Blot -Reported in scientific literature (PMID: 33202721).
• Application Notes: For IHC-Paraffin, HIER pH 6 retrieval is recommended. ICC/IF Fixation Permeabilization: Use PFA/Triton X-100.
• Control Peptide:

  FLNC Recombinant Protein Antigen (NBP1-89300PEP)

Packaging, Storage & Formulations

• Storage: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: PBS (pH 7.2) and 40% Glycerol
• Preservative: 0.02% Sodium Azide
• Purity: Immunogen affinity purified

Alternate Names for FLNC Antibody

• ABP-280
• ABP280A
• ABP-280-like protein
• ABPA
• ABP-L
• ABPLABP-L, gamma filamin
• Actin-binding-like protein
• filamin 2
• filamin C, gamma (actin binding protein 280)
• filamin C, gamma
• filamin-2
• filamin-C
• FLJ10186
• FLN2actin binding protein 280
• FLNc
• FLN-C
• Gamma-filamin

Background

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

Limitations

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.

Publications for FLNC Antibody (NBP1-89300)(8)

Publications using NBP1-89300

• Yerra VG, Batchu SN, Kaur H et al. Pressure overload induces ISG15 to facilitate adverse ventricular remodeling and promote heart failure The Journal of clinical investigation 2023-05-01 [PMID: 37115698] (WB, IHC-P, Mouse)
• Wu T, Xu Y, Zhang L et al. Filamin C is Essential for mammalian myocardial integrity PLOS Genetics 2023-01-27 [PMID: 36706168] (WB)
• Knyazeva A, Khudiakov A, Vaz R et al. FLNC Expression Level Influences the Activity of TEAD-YAP/TAZ Signaling Nat Commun 2020-11-13 [PMID: 33202721] (WB, Human, Mouse)
• Robertson R, Conte TC, Dicaire MJ et al. BAG3P215L/KO Mice as a Model of BAG3P209L Myofibrillar Myopathy Am. J. Pathol. 2020-01-14 [PMID: 31953038]
• Kiselev A, Vaz R, Knyazeva A et al. De novo mutations in FLNC leading to early-onset restrictive cardiomyopathy and congenital myopathy Hum. Mutat. 2018-06-01 [PMID: 29858533] (Human)
• Wu T, Mu Y, Bogomolovas J et al. HSPB7 is indispensable for heart development by modulating actin filament assembly. Proc. Natl. Acad. Sci. U.S.A. 2017-10-23 [PMID: 29078393] (Mouse)
• Reinstein E, Gutierrez-Fernandez A, Tzur S et al. Congenital dilated cardiomyopathy caused by biallelic mutations in Filamin C. Eur. J. Hum. Genet. 2016-09-07 [PMID: 27601210] (IHC-P)
• Valdes-Mas R, Gutierrez-Fernandez A, Gomez J et al. Mutations in filamin C cause a new form of familial hypertrophic cardiomyopathy Nat Commun et al. 2014-10-29 [PMID: 25351925] (IHC-P, Human)
  
  Details:
  FLnC/Filamin C antibody used for IHC-P on heart tissue sectionsfrom human control subjects and three hypertrophic cardiomyopathy patien - tone caused by a mutation in MYH7, and two without mutations in sarcomeric genes or FLnC, revealed large nuclei and larger fibre diameter, but not filamin C aggregates. IHC revealed the presence of FLnC in the protein aggregate occurring in cardiomyocytes of patients with FLnC mutations (Figure 3c).

Review for FLNC Antibody (NBP1-89300) (1)

Average Rating: 4

(Based on 1 review)

We have 1 review tested in 1 species: Mouse.

reviewed by: Pankaj Pathak IHC-Fr Mouse 06/28/2021

Summary

• Application: Immunohistochemistry-Frozen
• Sample Tested: Mouse skeletal muscle,Skeletal muscle tissue
• Species: Mouse
• Lot: 0113767

Comments

• Comments: 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.

Bioinformatics

• Gene Symbol: FLNC