Recombinant Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)
![Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)](https://www.abcam.com/ps/products/215/ab215376/Images/ab215376-327184-anti-dysferlin-antibody-jai-1-49-3-immunohistochemistry.jpg "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)")
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [JAI-1-49-3] to Dysferlin - Low endotoxin, Azide free
- Suitable for: WB, IHC-P, ICC/IF, IHC-Fr
- Reacts with: Mouse, Human
Related conjugates and formulations
Overview
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Product name
Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free
See all Dysferlin primary antibodies -
Description
Rabbit monoclonal [JAI-1-49-3] to Dysferlin - Low endotoxin, Azide free -
Host species
Rabbit -
Tested applications
Suitable for: WB, IHC-P, ICC/IF, IHC-Frmore details
Unsuitable for: Flow Cyt -
Species reactivity
Reacts with: Mouse, Human -
Immunogen
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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Positive control
- WB: Human and mouse skeletal muscle tissue lysates. IHC-P: Human skeletal muscle tissue. IHC-Fr: Human and mouse skeletal muscle tissues. ICC/IF: A673 cells.
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General notes
ab215376 is the carrier-free version of ab124684.
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 carrier-free antibodies are typically supplied in a PBS-only formulation, purified and free of BSA, sodium azide and glycerol. The carrier-free buffer and high concentration allow for increased conjugation efficiency.
This conjugation-ready format is designed for use with fluorochromes, metal isotopes, oligonucleotides, and enzymes, which makes them ideal for antibody labelling, functional and cell-based assays, flow-based assays (e.g. mass cytometry) and Multiplex Imaging applications.
Use our conjugation kits for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.
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 production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Our Low endotoxin, azide-free formats have low endotoxin level (≤ 1 EU/ml, determined by the LAL assay) and are free from azide, to achieve consistent experimental results in functional assays.
Rat: We have preliminary internal testing data to indicate this antibody may not react with this species. Please contact us for more information.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C. Do Not Freeze. -
Dissociation constant (KD)
KD = 7.20 x 10 -11 M Learn more about KD -
Storage buffer
pH: 7.20
Constituent: PBS -
Carrier free
Yes -
Concentration information loading... -
Purity
Protein A purified -
Clonality
Monoclonal -
Clone number
JAI-1-49-3 -
Isotype
IgG -
Research areas
Associated products
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Alternative Versions
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Compatible Secondaries
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Conjugation kits
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Isotype control
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Positive Controls
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab215376 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Abreviews | Notes |
|---|---|---|
| WB |
Use at an assay dependent concentration.
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| IHC-P |
Use at an assay dependent concentration. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
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| ICC/IF |
Use at an assay dependent concentration.
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| IHC-Fr |
Use at an assay dependent concentration.
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| Notes |
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WB
Use at an assay dependent concentration. |
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IHC-P
Use at an assay dependent concentration. Perform heat mediated antigen retrieval before commencing with IHC staining protocol. |
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ICC/IF
Use at an assay dependent concentration. |
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IHC-Fr
Use at an assay dependent concentration. |
Target
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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. -
Tissue specificity
Expressed in skeletal muscle, myoblast, myotube and in the syncytiotrophoblast (STB) of the placenta (at protein level). Highly expressed in skeletal muscle. Also found in heart, brain, spleen, intestine, placenta and at lower levels in liver, lung, kidney and pancreas. -
Involvement in disease
Defects in DYSF are the cause of limb-girdle muscular dystrophy type 2B (LGMD2B) [MIM:253601]. LGMD2B is an autosomal recessive degenerative myopathy characterized by weakness and atrophy starting in the proximal pelvifemoral muscles, with onset in the late teens or later, massive elevation of serum creatine kinase levels and slow progression. Scapular muscle involvement is minor and not present at onset. Upper limb girdle involvement follows some years after the onset in lower limbs.
Defects in DYSF are the cause of Miyoshi muscular dystrophy type (MMD1) [MIM:254130]. MMD1 is a late-onset muscular dystrophy involving the distal lower limb musculature. It is characterized by weakness that initially affects the gastrocnemius muscle during early adulthood. Otherwise the phenotype overlaps with LGMD2B, especially in age at onset and creatine kinase elevation.
Defects in DYSF are the cause of distal myopathy with anterior tibial onset (DMAT) [MIM:606768]. Onset of the disorder is between 14 and 28 years of age and the anterior tibial muscles are the first muscle group to be involved. Inheritance is autosomal recessive. -
Sequence similarities
Belongs to the ferlin family.
Contains 5 C2 domains. -
Developmental stage
Expression in limb tissue from 5-6 weeks embryos; persists throughout development. -
Domain
The C2 domain 1 associates with lipid membranes in a calcium-dependent manner. -
Cellular localization
Cell membrane > sarcolemma. Cytoplasmic vesicle membrane. Colocalizes, during muscle differentiation, with BIN1 in the T-tubule system of myotubules and at the site of contact between two myotubes or a myoblast and a myotube. Wounding of myotubes led to its focal enrichment to the site of injury and to its relocalization in a Ca(2+)-dependent manner toward the plasma membrane. Colocalizes with AHNAK, AHNAK2 and PARVB at the sarcolemma of skeletal muscle. Detected on the apical plasma membrane of the syncytiotrophoblast. Reaches the plasmma membrane through a caveolin-independent mechanism. Retained by caveolin at the plasmma membrane (By similarity). Colocalizes, during muscle differentiation, with CACNA1S in the T-tubule system of myotubules (By similarity). Accumulates and colocalizes with fusion vesicles at the sarcolemma disruption sites. - Information by UniProt
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Database links
- Entrez Gene: 8291 Human
- Entrez Gene: 26903 Mouse
- Omim: 603009 Human
- SwissProt: O75923 Human
- SwissProt: Q9ESD7 Mouse
- Unigene: 252180 Human
- Unigene: 220982 Mouse
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Alternative names
- DMAT antibody
- DYSF antibody
- DYSF_HUMAN antibody
see all
Images
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)
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. 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.
This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab124684).
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![Immunocytochemistry/ Immunofluorescence - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)](https://www.abcam.com/ps/products/215/ab215376/Images/ab215376-327183-anti-dysferlin-antibody-jai-1-49-3-immunofluorescence.jpg)
Immunocytochemistry/ Immunofluorescence - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)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. 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. ab7291, a mouse anti-tubulin (1/1000) and ab150120, an Alexa Fluor® 594-conjugated goat anti-mouse IgG (1/500) were also used.
Control 1: primary antibody (1/300) and secondary antibody, ab150120, an Alexa Fluor® 594-conjugated goat anti-mouse IgG (1/500).
Control 2: ab7291 (1/1000) and secondary antibody, ab150077, an Alexa Fluor® 488-conjugated goat anti-rabbit IgG (1/500).
This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab124684).
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![Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)](https://www.abcam.com/ps/products/215/ab215376/Images/ab215376-327182-anti-dysferlin-antibody-jai-1-49-3-immunohistochemistry.jpg)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human skeletal muscle tissue labelling Dysferlin with unpurified ab124684 at a dilution of 1/50.
This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab124684).
Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
-
![Immunohistochemistry (Frozen sections) - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)](https://www.abcam.com/ps/products/215/ab215376/Images/ab215376-327181-anti-dysferlin-antibody-jai-1-49-3-immunohistochemistry.jpg)
Immunohistochemistry (Frozen sections) - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)Immunohistochemistry (Frozen sections) analysis of unfixed frozen human skeletal muscle tissue (control) labelling Dysferlin with unpurified ab124684 at a dilution of 1/200.
This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab124684).
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![Immunohistochemistry (Frozen sections) - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)](https://www.abcam.com/ps/products/215/ab215376/Images/ab215376-327180-anti-dysferlin-antibody-jai-1-49-3-immunohistochemistry.jpg)
Immunohistochemistry (Frozen sections) - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)Immunohistochemistry (Frozen sections) analysis of unfixed frozen mouse skeletal muscle tissue (wild type) labelling Dysferlin with unpurified ab124684 at a dilution of 1/200.
This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab124684).
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![OI-RD Scanning - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)](https://www.abcam.com/ps/products/215/ab215376/Images/ab215376-327179-anti-dysferlin-antibody-jai-1-49-3-.jpg)
OI-RD Scanning - Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)Equilibrium disassociation constant (KD)
Learn more about KD
Click here to learn more about KDThis data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab124684).
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![Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)](https://www.abcam.com/ps/products/215/ab215376/Images/ab215376-7-benefits-of-recombinant-antibodies.png)
Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)
Protocols
Datasheets and documents
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Datasheet download
Certificate of Compliance
References (7)
ab215376 has been referenced in 7 publications.
- Kim SC et al. TRIM72 is required for effective repair of alveolar epithelial cell wounding. Am J Physiol Lung Cell Mol Physiol 307:L449-59 (2014). PubMed: 25106429
- Defour A et al. Dysferlin regulates cell membrane repair by facilitating injury-triggered acid sphingomyelinase secretion. Cell Death Dis 5:e1306 (2014). ICC/IF ; Human . PubMed: 24967968
- Tzeng HP et al. Dysferlin mediates the cytoprotective effects of TRAF2 following myocardial ischemia reperfusion injury. J Am Heart Assoc 3:e000662 (2014). WB ; Mouse . PubMed: 24572254
- Kerr JP et al. Dysferlin stabilizes stress-induced Ca2+ signaling in the transverse tubule membrane. Proc Natl Acad Sci U S A 110:20831-6 (2013). ICC/IF ; Mouse . PubMed: 24302765
- Lek A et al. Calpains, cleaved mini-dysferlinC72, and L-type channels underpin calcium-dependent muscle membrane repair. J Neurosci 33:5085-94 (2013). WB ; Human . PubMed: 23516275
- Azakir BA et al. Proteasomal inhibition restores biological function of mis-sense mutated dysferlin in patient-derived muscle cells. J Biol Chem 287:10344-54 (2012). PubMed: 22318734
- Wang ZQ et al. Distinguishing the 4qA and 4qB variants is essential for the diagnosis of facioscapulohumeral muscular dystrophy in the Chinese population. Eur J Hum Genet 19:64-9 (2011). WB ; Human . PubMed: 20736973
