Recombinant
RabMAb

Recombinant Anti-Dysferlin antibody [JAI-1-49-3] - Low endotoxin, Azide free (ab215376)

Overview

  • 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 (the amino acid sequence is considered to be commercially sensitive) corresponding to Human Dysferlin aa 100-200.
    Database link: O75923

  • 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.
  • General notes

    ab215376 is a carrier-free antibody designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.

     

    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.

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

    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 rabbit monoclonal antibody.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C. Avoid freeze / thaw cycle.
  • Dissociation constant (KD)

    KD = 7.20 x 10 -11 M
    Learn more about KD
  • Storage buffer

    pH: 7.20
    Constituent: PBS
  • Concentration information loading...
  • Purity

    Protein A purified
  • Clonality

    Monoclonal
  • Clone number

    JAI-1-49-3
  • Isotype

    IgG
  • Research areas

Applications

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. Detects a band of approximately 280 kDa (predicted molecular weight: 237 kDa).
IHC-P Use at an assay dependent concentration. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.

See IHC antigen retrieval protocols.

ICC/IF Use at an assay dependent concentration.

 

IHC-Fr Use at an assay dependent concentration.
  • Application notes
    Is unsuitable for Flow Cyt.
  • Target

    • 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
    • Database links

    • Alternative names

      • DMAT antibody
      • DYSF antibody
      • DYSF_HUMAN antibody
      • Dysferlin antibody
      • Dysferlin limb girdle muscular dystrophy 2B (autosomal recessive) antibody
      • Dysferlin limb girdle muscular dystrophy 2B antibody
      • Dystrophy associated fer 1 like 1 antibody
      • Dystrophy associated fer 1 like protein antibody
      • Dystrophy associated fer1 like 1 antibody
      • Dystrophy associated fer1 like protein antibody
      • Dystrophy-associated fer-1-like protein antibody
      • Fer 1 like protein 1 antibody
      • Fer-1-like protein 1 antibody
      • Fer1 like protein 1 antibody
      • FER1L1 antibody
      • FLJ00175 antibody
      • FLJ90168 antibody
      • LGMD 2B antibody
      • LGMD2B antibody
      • Limb girdle muscular dystrophy 2B (autosomal recessive) antibody
      • Limb girdle muscular dystrophy 2B antibody
      • Miyoshi myopathy antibody
      • MM antibody
      • MMD1 antibody
      see all

    Images

    • 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).

    • 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).

    • 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) 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).

    • 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).

    • Equilibrium disassociation constant (KD)
      Learn more about KD

      Click here to learn more about KD

      This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab124684).

    References

    This product has been referenced in:

    • 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). Read more (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 . Read more (PubMed: 24967968) »
    See all 7 Publications for this product

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