Name: Anti-Dysferlin antibody [JAI-1-49-3]
SKU: ab124684
Rating: 4 (4 reviews)

Rabbit Recombinant Monoclonal Dysferlin antibody. Suitable for IHC-P, WB, ICC/IF, IHC-Fr and reacts with Human, Mouse samples. Cited in 38 publications.

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Images

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dysferlin antibody [JAI-1-49-3] (AB124684), expandable thumbnail

Publications

Key facts

Isotype: IgG
Host species: Rabbit
Storage buffer: pH: 7.2 - 7.4
Preservative: 0.01% Sodium azide
Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA
Form: Liquid
Clonality: Monoclonal

Immunogen

The exact immunogen used to generate this antibody is proprietary information.

Reactivity data

Select an application

Product promiseTestedExpectedPredictedNot recommended

	IHC-P	Flow Cyt	WB	ICC/IF	IHC-Fr
Human	Tested	Not recommended	Tested	Tested	Tested
Mouse	Expected	Not recommended	Tested	Expected	Tested

Tested
Tested

Species	Dilution info	Notes
Species Human	Dilution info 1/500	Notes For unpurified use at 1/50 - 1/100. Perform heat-mediated antigen retrieval before commencing with IHC staining protocol.

Expected
Expected

Species	Dilution info	Notes
Species Mouse	Dilution info Use at an assay dependent concentration.	Notes -

Not recommended
Not recommended

Species	Dilution info	Notes
Species Mouse, Human	Dilution info -	Notes -

Tested
Tested

Species	Dilution info	Notes
Species Mouse	Dilution info 1/1000	Notes For unpurified use at 1/1000 - 1/10000.
Species Human	Dilution info 1/1000	Notes For unpurified use at 1/1000 - 1/10000.

Tested
Tested

Species	Dilution info	Notes
Species Human	Dilution info 1/200 - 1/400	Notes -

Expected
Expected

Species	Dilution info	Notes
Species Mouse	Dilution info Use at an assay dependent concentration.	Notes -

Tested
Tested

Species	Dilution info	Notes
Species Mouse	Dilution info 1/200	Notes -
Species Human	Dilution info 1/200	Notes -

Associated Products

Select an associated product type

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1 product for Alternative Version

Target data

See full target information DYSF

Function

Key calcium ion sensor involved in the Ca(2+)-triggered synaptic vesicle-plasma membrane fusion. Plays a role in the sarcolemma repair mechanism of both skeletal muscle and cardiomyocytes that permits rapid resealing of membranes disrupted by mechanical stress (By similarity).

Alternative names

FER1L1, DYSF, Dysferlin, Dystrophy-associated fer-1-like protein, Fer-1-like protein 1

Recommended products

Rabbit Recombinant Monoclonal Dysferlin antibody. Suitable for IHC-P, WB, ICC/IF, IHC-Fr and reacts with Human, Mouse samples. Cited in 38 publications.

Key facts

Isotype: IgG
Host species: Rabbit
Storage buffer: pH: 7.2 - 7.4
Preservative: 0.01% Sodium azide
Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA
Form: Liquid
Clonality: Monoclonal
Immunogen: The exact immunogen used to generate this antibody is proprietary information.
Clone number: JAI-1-49-3
Purification technique: Affinity purification Protein A
Dissociation constant: 7.2 x 10^-11 M
Concentration

Storage

Shipped at conditions: Blue Ice
Appropriate short-term storage duration: 1-2 weeks
Appropriate short-term storage conditions: +4°C
Appropriate long-term storage conditions: -20°C
Aliquoting information: Upon delivery aliquot
Storage information: Avoid freeze / thaw cycle

Notes

This antibody was made in collaboration with the Jain Foundation whose goal is to hasten EVERY avenue that may lead to the cure for LGMD2B/Miyoshi.

Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.

This product is a recombinant monoclonal antibody, which offers several advantages including:

- High batch-to-batch consistency and reproducibility
- Improved sensitivity and specificity
- Long-term security of supply
- Animal-free batch production

For more information, read more on recombinant antibodies.

Rat: We have preliminary internal testing data to indicate this antibody may not react with this species. Please contact us for more information.

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Dysferlin also known by its alternate names DYSF and CAPN3 is a protein characterized by a mass of approximately 237 kilodaltons. It belongs to the ferlin family and is mainly expressed in skeletal and cardiac muscle tissues. This protein contains multiple C2 domains which are significant for its membrane-trafficking roles. Dysferlin has an important role at the muscle surface particularly in the repair of sarcolemma breaches which helps maintain muscle cell integrity.

Biological function summary

The action of dysferlin impacts several molecular processes critical for muscle function and maintenance often interacting as part of a larger protein complex. It facilitates membrane repair by promoting vesicle fusion events and recruits other proteins to damaged sites. Such action is vital in restoring cellular structure after membrane damage particularly under the mechanical stress experienced in muscle tissues.

Pathways

Dysferlin operates within the calcium-dependent membrane repair pathway and the broader muscular dystrophy pathway. It interacts importantly with proteins such as annexins and actin that aid in membrane repair processes. Dysferlin's role in cellular repair connects it to pathways related to muscle membrane dynamics and stress response further highlighting its function in muscle health and recovery.

Associated diseases and disorders

Mutations or deficiencies in dysferlin are linked to muscular dystrophies such as limb-girdle muscular dystrophy type 2B and Miyoshi myopathy. These conditions are characterized by progressive muscle weakness and wasting. The link between dysferlin's function and these disorders is also seen in its association with proteins like calpain 3 which also plays a role in muscle maintenance. Understanding the line of interaction between dysferlin and these proteins offers insights into potential therapeutic targets for these conditions.

Product promise

We are dedicated to supporting your work with high quality reagents and we are here for you every step of the way should you need us.

In the unlikely event of one of our products not working as expected, you are covered by our product promise.

Full details and terms and conditions can be found here:
Terms & Conditions.

10 product images

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human skeletal muscle tissue labelling Dysferlin with purified ab124684 at 1/500. Heat mediated antigen retrieval was performed using Tris/EDTA buffer pH 9. Goat Anti-Rabbit IgG H&L (HRP) ab97051, a HRP-conjugated goat anti-rabbit IgG (H+L) was used as the secondary antibody (1/500). Negative control using PBS instead of primary antibody. Counterstained with hematoxylin.
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human skeletal muscle tissue labelling Dysferlin with unpurified ab124684 at a dilution of 1/50.
Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
Immunohistochemistry (Frozen sections) - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)
Immunohistochemistry (Frozen sections) analysis of unfixed frozen human skeletal muscle tissue (control) labelling Dysferlin with unpurified ab124684 at a dilution of 1/200.
Immunocytochemistry/ Immunofluorescence - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)
Immunocytochemistry/Immunofluorescence analysis of A673 cells labelling Dysferlin with purified ab124684 at 1/300. Cells were fixed with 4% paraformaldehyde and permeabilized with 0.1% Triton X-100. Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) ab150077, an Alexa Fluor^® 488-conjugated goat anti-rabbit IgG (1/500) was used as the secondary antibody. DAPI (blue) was used as the nuclear counterstain. Anti-alpha Tubulin antibody [DM1A] - Loading Control ab7291, a mouse anti-tubulin (1/1000) and Goat Anti-Mouse IgG H&L (Alexa Fluor® 594) preadsorbed ab150120, an Alexa Fluor^® 594-conjugated goat anti-mouse IgG (1/500) were also used.
Control 1: primary antibody (1/300) and secondary antibody, Goat Anti-Mouse IgG H&L (Alexa Fluor® 594) preadsorbed ab150120, an Alexa Fluor^® 594-conjugated goat anti-mouse IgG (1/500).
Control 2: Anti-alpha Tubulin antibody [DM1A] - Loading Control ab7291 (1/1000) and secondary antibody, Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) ab150077, an Alexa Fluor^® 488-conjugated goat anti-rabbit IgG (1/500).
Western blot - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)
Blocking and dilution buffer: 5% NFDM/TBST.
All lanes: Western blot - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684) at 1/1000 dilution
All lanes: Mouse muscle tissue lysate at 10 µg
Secondary
All lanes: Peroxidase-conjugated goat anti-rabbit IgG, (H+L) at 1/1000 dilution
Predicted band size: 237 kDa
Observed band size: 280 kDa
Western blot - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)
Blocking and dilution buffer: 5% NFDM/TBST.
All lanes: Western blot - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684) at 1/1000 dilution
All lanes: Human skeletal muscle tissue lysate at 20 µg
Secondary
All lanes: Peroxidase-conjugated goat anti-rabbit IgG, (H+L) at 1/1000 dilution
Predicted band size: 237 kDa
Observed band size: 280 kDa
Western blot - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)
All lanes: Western blot - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684) at 1/1000 dilution
Lane 1: Human skeletal muscle tissue lysate (control) at 10 µg
Lane 2: Human skeletal muscle tissue lysate (LGMD2B) at 10 µg
Lane 3: Mouse skeletal muscle tissue lysate (wild-type mice) at 10 µg
Lane 4: Mouse skeletal muscle tissue lysate (Dysf-/- transgenic mouse) at 10 µg
Secondary
All lanes: HRP-conjugated goat anti-rabbit IgG at 1/2000 dilution
Predicted band size: 237 kDa
Observed band size: 280 kDa
Immunohistochemistry (Frozen sections) - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)
Immunohistochemistry (Frozen sections) analysis of unfixed frozen mouse skeletal muscle tissue (wild type) labelling Dysferlin with unpurified ab124684 at a dilution of 1/200.
OI-RD Scanning - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)
We have systematically measured KD (the equilibrium dissociation constant between the antibody and its antigen), of more than 840 recombinant antibodies to assess not only their individual KD values but also to see the average affinity of antibody.
Based on the comparison with published literature values for mouse monoclonal antibodies, Recombinant antibodies appear to be on average 1-2 order of magnitude higher affinity.
Image collected and cropped by CiteAb under a CC-BY license from the publication
Western blot - Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)
Dysferlin western blot using anti-Dysferlin antibody [JAI-1-49-3] ab124684. Publication image and figure legend from Vanhoutte, D., Schips, T. G., et al., 2016, Elife, PubMed 27669143.

ab124684 was used in this publication in western blot. This may not be the same as the application(s) guaranteed by Abcam. For a full list of applications guaranteed by Abcam for ab124684 please see the product overview.
Thbs4 enhances stabilizing proteins at the sarcolemma.(A) Immunofluoresence (green) detection of δ-, α-, β-, and γ-sarcoclycan (SGC), β-dystroglycan (β-DG), utrophin (Utro.), dystrophin (Dystro.) and β1D-integrin in littermates of three month-old WT, Thbs4-Tg, Sgcd-/- and Sgcd-/-Thbs4-Tg quadriceps. Representative images of 4 mice per genotype are shown. Scale bar = 25 μm. (B) Representative Western blots of sarcolemmal protein extracts (upper) or total cytoplasmic protein extracts (lower) from the quadriceps of the indicated groups of mice for the indicated proteins (n = 4–5 biological replicates). Abbreviations: Utro, utrophin; Dystro, dystrophin; Dysfer, dysferlin; α-DG, α-dystroglycan; β-DG, β-dystroglycan; δ-SCG, δ-sarcoglycan; α-SCG, α-sarcoglycan; β-SGC, β-sarcoglycan; β1D-, α7- and α5- integrin. The red boxes show increased protein levels. Also see Figure 6—figure supplement 1 for replicates. (C) Representative immunoblot for structural components of the DGC- and integrin-associated protein complexes in sarcolemmal preparations from Thbs4-/- and WT quadriceps at four months of age (n = 4 biological replicates). The burgundy-boxed areas show reduced protein levels. Ponceau staining of a nonspecific band and dihydropyridine receptor α1 (Cav1.1) were used as loading controls for sarcolemmal protein extracts; Gapdh was used as loading control for total cell protein extracts.DOI:http://dx.doi.org/10.7554/eLife.17589.018Thbs4 enhances stabilizing proteins at the sarcolemma and directly interacts with integrins.(A,B) Representative Western blots of sarcolemmal protein extracts from the quadriceps of the indicated groups of mice at three months of age (n = 4–5 biological replicates). Sgcd-/- Tg and mdx Tg indicate Sgcd-/- and mdx with skeletal muscle specific Thbs4 overexpression, respectively. Ponceau staining of a nonspecific band and dihydropyridine receptor α1 (Cav1.1) were used as loading controls. Abbreviations: Utro, utrophin; Dystro, dystrophin; Dysfer, dysferlin; α-DG, α-dystroglycan; β-DG, β-dystroglycan; δ-SCG, δ-sarcoglycan; α-SCG, α-sarcoglycan; β-SGC, β-sarcoglycan; β1D-, α7- and α5-itg (integrin). (C) Immunoblots for β1D- and α7-Integrin (Itg), β-dystroglycan (DG) and Thbs4 (Flag) from neonatal rat ventricular myocyte extracts immunoprecipitated with a Flag antibody (Thbs4). Adβgal was used as a control infection (n = 3 biological replicates). An adenovirus expressing a Flag-tagged Thbs4 protein was used to achieve high level of this protein to identify the interaction. (D,E) Representative Western blots for Thbs4, α5- and β1D-integrin (itg) from intracellular vesicular isolates from WT and Thbs4 Tg quadriceps (D) or WT and Sgcd-/-quadriceps (E) that were immunoprecipitated with an antibody raised against the cytoplasmic domain of β1D-integrin (n = 3 biological replicates), showing that Thbs4 and α5-integrin localize to β1D-integrin-positive intracellular vesicles. α-tubulin and Gapdh are presented as loading control.DOI:http://dx.doi.org/10.7554/eLife.17589.019