Overview

  • Product name

    Anti-Dysferlin antibody - N-terminal
    See all Dysferlin primary antibodies
  • Description

    Rabbit polyclonal to Dysferlin - N-terminal
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, ICC/IF, IPmore details
  • Species reactivity

    Reacts with: Mouse, Human
  • Immunogen

    Synthetic peptide within Human Dysferlin (N terminal) conjugated to Keyhole Limpet Haemocyanin (KLH). The exact sequence is proprietary.
    Database link: O75923

  • Positive control

    • HEK293T cell lysate overexpressing Human Dysferlin; Differentiated C2C12 myoblast cells

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 long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.40
    Preservative: 0.098% Sodium azide
    Constituent: 99% PBS
  • Concentration information loading...
  • Purity

    Affinity purified
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab214063 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 a concentration of 0.5 - 1 µg/ml. Predicted molecular weight: 237 kDa.
ICC/IF Use a concentration of 7 - 14 µg/ml.
IP Use at an assay dependent concentration.

Use 5 - 10 μg per reaction.

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

  • All lanes : Anti-Dysferlin antibody - N-terminal (ab214063) at 0.5 µg/ml

    Lane 1 : HEK293T cell lysate overexpressing Human Dysferlin
    Lane 2 : HEK293T cell lysate non-transfected
    Lane 3 : HEK293T cell lysate overexpressing Human Dysferlin with immunizing peptide

    Secondary
    All lanes : Goat Anti-Rabbit IgG-Peroxidase

    Developed using the ECL technique.

    Predicted band size: 237 kDa

  • Immunofluorescent analysis of fixed, differentiated C2C12 myoblast cells cells labeling Dysferlin with ab214063 at 10 µg/mL, followed by Goat Anti-Rabbit IgG, Cy3 conjugate and nuclear staining with Hoescht 33342 (blue).

  • ab214063 was used to immunoprecipitate Dysferlin from HEK293T cells overexpressing human Dysferlin.

    For WB detection a different antibody anti-Dysferlin was used.


    Lanes
    1. IP antibody (ab214063): 5 μg
    2. Negative control: without IP antibody
    3. Negative control: without cell lysate
    4. WB control, Detection antibody: ab214063

References

ab214063 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Application
Immunohistochemistry (Frozen sections)
Sample
Mouse Tissue sections (Muscle)
Permeabilization
Yes - 2 min Triton 1%
Specification
Muscle
Blocking step
BSA as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 25°C
Fixative
Paraformaldehyde

Miss. Nathalie Da Silva

Verified customer

Submitted Feb 03 2017

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