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Dysferlin Antibody

Images

 
Immunocytochemistry/ Immunofluorescence: Dysferlin Antibody [NBP1-84696] - Staining of human cell line RH-30 shows localization to plasma membrane & microtubule organizing center.
Immunohistochemistry-Paraffin: Dysferlin Antibody [NBP1-84696] - Staining of human testis shows moderate membranous positivity in cells in seminiferous ducts.
Immunohistochemistry-Paraffin: Dysferlin Antibody [NBP1-84696] - Staining of human skin shows low expression as expected.
Orthogonal Strategies: Immunohistochemistry-Paraffin: Dysferlin Antibody [NBP1-84696] - Analysis in human skeletal muscle and skin tissues. Corresponding Dysferlin RNA-seq data are presented for the same tissues.
Independent Antibodies: Immunohistochemistry-Paraffin: Dysferlin Antibody [NBP1-84696] - Staining of human kidney, skeletal muscle, skin and testis using Anti-Dysferlin antibody NBP1-84696 (A) shows similar ...read more
Immunohistochemistry-Paraffin: Dysferlin Antibody [NBP1-84696] - Staining of human kidney shows moderate to strong membranous positivity in cells in glomeruli and cells in tubules.
Immunohistochemistry-Paraffin: Dysferlin Antibody [NBP1-84696] - Staining of human skeletal muscle shows strong cytoplasmic and membranous positivity in myocytes.

Product Details

Summary
Reactivity Hu, Mu, RtSpecies Glossary
Applications ICC/IF, IHC
Clonality
Polyclonal
Host
Rabbit
Conjugate
Unconjugated

Order Details

Dysferlin Antibody Summary

Immunogen
This antibody was developed against Recombinant Protein corresponding to amino acids: IVVELYDHDTYGADEFMGRCICQPSLERMPRLAWFPLTRGSQPSGELLASFELIQREKPAIHHIPGFEVQETSRILDESEDTDLPYPPPQREANIYMVPQNIKPALQRTAIEILAWG
Predicted Species
Mouse (96%), Rat (96%). Backed by our 100% Guarantee.
Isotype
IgG
Clonality
Polyclonal
Host
Rabbit
Gene
DYSF
Purity
Immunogen affinity 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 0.25-2 ug/ml
  • Immunohistochemistry 1:200 - 1:500
  • Immunohistochemistry-Paraffin 1:200 - 1:500
Application Notes
For IHC-Paraffin, HIER pH 6 retrieval is recommended. ICC/IF Fixation Permeabilization: Use PFA/Triton X-100.
Control Peptide
Dysferlin Protein (NBP1-84696PEP)

Packaging, Storage & Formulations

Storage
Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
Buffer
PBS (pH 7.2) and 40% Glycerol
Preservative
0.02% Sodium Azide
Purity
Immunogen affinity purified

Alternate Names for Dysferlin Antibody

  • 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

ICC/IF Video Protocol

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Secondary Antibodies

 

Isotype Controls

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Bioinformatics

Gene Symbol DYSF