FLNC Antibody

Images

 
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.

Product Details

Summary
Reactivity Hu, Mu, RtSpecies Glossary
Applications WB, ICC/IF, IHC, IHC-Fr, IHC-P
Clonality
Polyclonal
Host
Rabbit
Conjugate
Unconjugated
Validated by:
       

Orthogonal Strategies

 

Order Details

FLNC Antibody Summary

Immunogen
This antibody was developed against Recombinant Protein corresponding to amino acids: HSLHETSTVLVETVTKSSSSRGSSYSSIPKFSSDASKVVTRGPGLSQAFVGQKNSFTVDCSKAGTNMMMVGVHGPKTPCEEVYVKHMGNRVYNVTYTVKEKGDY
Predicted Species
Rat (99%). Backed by our 100% Guarantee.
Isotype
IgG
Clonality
Polyclonal
Host
Rabbit
Gene
FLNC
Purity
Immunogen affinity purified
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.

Applications/Dilutions

Dilutions
  • Immunocytochemistry/Immunofluorescence 0.25 - 2 ug/mL
  • Immunohistochemistry 1:200 - 1:500
  • Immunohistochemistry-Frozen
  • Immunohistochemistry-Paraffin 1:200 - 1:500
  • Western Blot
Application Notes
Use in WB reported in scientific literature (PMID: 33202721). For IHC-Paraffin, HIER pH 6 retrieval is recommended. ICC/IF Fixation Permeabilization: Use PFA/Triton X-100. FLNC Antibody is validated for IHC-Fr from a verified customer review.
Control Peptide
FLNC Recombinant Protein Antigen (NBP1-89300PEP)
Reviewed Applications
Read 1 Review rated 4
using
NBP1-89300 in the following applications:

Publications
Read Publications using
NBP1-89300 in the following applications:

  • 2 publications
  • WB
    1 publication

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.

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Applications: WB, ICC/IF, IHC, IHC-Fr, IHC-P

Publications for FLNC Antibody (NBP1-89300)(6)

We have publications tested in 2 confirmed species: Human, Mouse.

We have publications tested in 2 applications: IHC-P, WB.


Filter By Application
IHC-P
(2)
WB
(1)
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Human
(3)
Mouse
(2)
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Showing Publications 1 - 6 of 6.
Publications using NBP1-89300 Applications Species
Knyazeva A, Khudiakov A, Vaz R et al. FLNC Expression Level Influences the Activity of TEAD-YAP/TAZ Signaling Nat Commun Nov 13 2020 [PMID: 33202721] (WB, Human, Mouse) WB Human, Mouse
Robertson R, Conte TC, Dicaire MJ et al. BAG3P215L/KO Mice as a Model of BAG3P209L Myofibrillar Myopathy Am. J. Pathol. Jan 14 2020 [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. Jun 1 2018 [PMID: 29858533] (Human) 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 Oct 23 [PMID: 29078393] (Mouse) Mouse
Reinstein E, Gutierrez-Fernandez A, Tzur S et al. Congenital dilated cardiomyopathy caused by biallelic mutations in Filamin C. Eur. J. Hum. Genet. Sep 7 2016 [PMID: 27601210] (IHC-P) 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 Oct 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).
IHC-P Human

Review for FLNC Antibody (NBP1-89300) (1) 41

Average Rating: 4
(Based on 1 review)
We have 1 review tested in 1 species: Mouse.

Reviews using NBP1-89300:
Filter by Applications
IHC-Fr
(1)
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Filter by Species
Mouse
(1)
All Species
Images Ratings Applications Species Date Details
Immunohistochemistry-Frozen FLNC NBP1-89300
Enlarge
4
reviewed by:
Verified Customer
IHC-Fr Mouse 06/28/2021
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Summary

ApplicationImmunohistochemistry-Frozen
Sample TestedMouse skeletal muscle,Skeletal muscle tissue
SpeciesMouse
Lot0113767

Comments

CommentsPathak, 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.

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ICC/IF Video Protocol

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Recent Reviews

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Verified Customer
06/28/2021
Application: IHC-Fr
Species: Mouse

Bioinformatics

Gene Symbol FLNC