Overview

  • Product name
  • Description
    Rabbit polyclonal to Dysferlin
  • Tested applications
    Suitable for: WB, IHC-P, ICC/IFmore details
  • Species reactivity
    Reacts with: Dog, Human
    Predicted to work with: Mouse
  • Immunogen

    Synthetic peptide (unfortunately, the amino acid sequence is considered to be commercially sensitive) (Human) (C terminal).

  • Positive control
    • Skeletal muscle.

Properties

  • Form
    Liquid
  • Storage instructions
    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • Storage buffer
    Preservative: 0.1% Sodium Azide
    Constituents: 1% BSA, 10mM PBS, pH 7.4
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab15108 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 1 µg/ml. Predicted molecular weight: 231 kDa.
IHC-P 1/50.
ICC/IF Use at an assay dependent concentration.

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

  • ab15108 staining Dysferlin in human skeletal muscle by Immunohistochemistry (FFPE-sections).

  • Anti-Dysferlin antibody (ab15108) at 1 µg/ml + Human skeletal muscle tissue lysate - total protein (ab29330) at 10 µg

    Secondary
    Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution

    Predicted band size : 231 kDa
    Observed band size : 231 kDa
    Additional bands at : 240 kDa (possible post-translational modification).

References

This product has been referenced in:
  • Whish S  et al. The inner CSF-brain barrier: developmentally controlled access to the brain via intercellular junctions. Front Neurosci 9:16 (2015). IHC . Read more (PubMed: 25729345) »
  • Guo LT  et al. Evaluation of commercial dysferlin antibodies on canine, mouse and human skeletal muscle. Neuromuscul Disord : (2010). WB ; Human, Mouse, Dog . Read more (PubMed: 20817457) »

See all 4 Publications for this product

Customer reviews and Q&As

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

Miss. Nathalie Da Silva

Verified customer

Submitted Feb 09 2017

Application
Western blot
Loading amount
50 µg
Gel Running Conditions
Reduced Denaturing (4-12% Bis-Tris gel)
Sample
Sheep Tissue lysate - whole (Right atrial appendage)
Specification
Right atrial appendage
Blocking step
SuperBlock as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 100% · Temperature: 22°C
Username

Abcam user community

Verified customer

Submitted Apr 03 2014

Thank you for submitting an Abreview for this product.

I am sorry to see that this product did not perform as expected in WB on canine and human samples. We guarantee our products to work as stated on the datasheet and would appreciate the o...

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Abreviews
Application
Western blot
Sample
Human Cell lysate - whole cell (HeLa)
Loading amount
25 µg
Specification
HeLa
Gel Running Conditions
Reduced Denaturing (Bio-Rad/TGX/Any kD)
Blocking step
Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Abcam user community

Verified customer

Submitted Dec 04 2012

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Immunocytochemistry/ Immunofluorescence
Sample
Dog Cell (Muscle)
Specification
Muscle
Fixative
Acetone
Username

Abcam user community

Verified customer

Submitted Jul 21 2008

Thank you for your enquiry. Since you are using 4% PFA, which also contains formalin, I would suggest doing the antigen retrieval step. Formaldehyde fixes not by coagulation, but by reacting primarily with basic amino acids to form cross-linking "m...

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Thank you for your enquiry. The antigen sequence is exactly same as that of mouse (100%). I hope this information helps. Please do not hesitate to contact us again if you need anything further.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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