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Dysferlin Antibody (JF05-54)

Images

 
Western Blot: Dysferlin Antibody (JF05-54) [NBP2-66814] - Analysis of Dysferlin on human skeletal muscle lysates using anti-Dysferlin antibody at 1/1,000 dilution.
Immunocytochemistry/ Immunofluorescence: Dysferlin Antibody (JF05-54) [NBP2-66814] - Staining Dysferlin in SW480 cells (red). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised ...read more
Immunohistochemistry-Paraffin: Dysferlin Antibody (JF05-54) [NBP2-66814] - Immunohistochemical analysis of paraffin-embedded human spleen tissue using anti-Dysferlin antibody. The section was pre-treated using heat ...read more
Immunohistochemistry-Paraffin: Dysferlin Antibody (JF05-54) [NBP2-66814] - Analysis of paraffin-embedded human spleen tissue using anti-Dysferlin antibody. Counter stained with hematoxylin.
Immunohistochemistry-Paraffin: Dysferlin Antibody (JF05-54) [NBP2-66814] - Analysis of paraffin-embedded mouse heart tissue using anti-Dysferlin antibody. Counter stained with hematoxylin.
Immunohistochemistry-Paraffin: Dysferlin Antibody (JF05-54) [NBP2-66814] - Analysis of paraffin-embedded mouse skeletal muscle tissue using anti-Dysferlin antibody. Counter stained with hematoxylin.
Immunohistochemistry-Paraffin: Dysferlin Antibody (JF05-54) [NBP2-66814] - Analysis of paraffin-embedded mouse spleen tissue using anti-Dysferlin antibody. Counter stained with hematoxylin.

Product Details

Summary
Reactivity Hu, MuSpecies Glossary
Applications WB, ICC/IF, IHC
Clone
JF05-54
Clonality
Monoclonal
Host
Rabbit
Conjugate
Unconjugated
Concentration
1 mg/ml

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Dysferlin Antibody (JF05-54) Summary

Additional Information
Recombinant Monoclonal Antibody.
Immunogen
Synthetic peptide within Human Dysferlin aa 113-162 / 2080. (SwissProt: O75923 Human; SwissProt: Q9ESD7 Mouse)
Localization
Cell membrane, Cytoplasmic vesicle membrane.
Isotype
IgG
Clonality
Monoclonal
Host
Rabbit
Gene
DYSF
Purity
Protein A purified
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.

Applications/Dilutions

Dilutions
  • Immunocytochemistry/ Immunofluorescence 1:50-1:200
  • Immunohistochemistry
  • Immunohistochemistry-Paraffin 1:50-1:200
  • Western Blot 1:1000-1:5000
Theoretical MW
237 kDa.
Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.

Packaging, Storage & Formulations

Storage
Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
Buffer
TBS (pH7.4), 0.05% BSA, 40% Glycerol
Preservative
0.05% Sodium Azide
Concentration
1 mg/ml
Purity
Protein A purified

Alternate Names for Dysferlin Antibody (JF05-54)

  • dysferlin
  • dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive)
  • dystrophy-associated fer-1-like 1
  • FER1L1Dystrophy-associated fer-1-like protein
  • Fer-1-like protein 1
  • FLJ00175
  • FLJ90168
  • LGMD2B

Background

Dysferlin is the protein product of the 2p13 gene that is defective in patients with Limb-Girdle Muscular Dystrophy type 2B (LGMD2B) and Miyoshi Myopathy (MM). Dysferlin is normally localized to the muscle plasma membrane. In patients with LGMD2B and MM, immunoreactivity to dysferlin is severely reduced or lost, depending on the type of mutation. This antibody is used for the characterization of LGMD2B and MM.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Product General Protocols

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Video Protocols

WB Video Protocol
ICC/IF Video Protocol

FAQs for Dysferlin Antibody (NBP2-66814) (0)

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Secondary Antibodies

 

Isotype Controls

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Bioinformatics

Gene Symbol DYSF