Dystrophin Recombinant antibody, PBS Only

Dystrophin Uni-rAb^TM Recombinant Antibody for IHC, IF-P, FC (Intra), ELISA

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse, rat

Applications

IHC, IF-P, FC (Intra), ELISA

Conjugate

Unconjugated

CloneNo.

240567C4

Cat no : 83609-6-PBS

Synonyms

DMD, BMD, 240567C4

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Validation Data Gallery

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  IHC staining of mouse skeletal muscle using 83609-6-RR (same clone as 83609-6-PBS)

  Immunohistochemical analysis of paraffin-embedded mouse skeletal muscle tissue slide using 83609-6-RR (Dystrophin antibody) at dilution of 1:200 (under 10x lens). Heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0). This data was developed using the same antibody clone with 83609-6-PBS in a different storage buffer formulation.

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  IF Staining of rat brain using 83609-6-RR (same clone as 83609-6-PBS)

  Immunofluorescent analysis of (4% PFA) fixed paraffin-embedded rat brain tissue using Dystrophin antibody (83609-6-RR, Clone: 240567C4 ) at dilution of 1:400 and Multi-rAb CoraLite ® Plus 488-Goat Anti-Rabbit Recombinant Secondary Antibody (H+L) (RGAR002). Heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0). This data was developed using the same antibody clone with 83609-6-PBS in a different storage buffer formulation.

• 

  IF Staining of rat brain using 83609-6-RR (same clone as 83609-6-PBS)

  Immunofluorescent analysis of (4% PFA) fixed paraffin-embedded rat brain tissue using Dystrophin antibody (83609-6-RR, Clone: 240567C4 ) at dilution of 1:400 and Multi-rAb CoraLite ® Plus 488-Goat Anti-Rabbit Recombinant Secondary Antibody (H+L) (RGAR002). Heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0). This data was developed using the same antibody clone with 83609-6-PBS in a different storage buffer formulation.

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  FC experiment of HepG2 using 83609-6-RR (same clone as 83609-6-PBS)

  1x10^6 HepG2 cells were intracellularly stained with 0.25 ug DMD Recombinant antibody (83609-6-RR, Clone:240567C4) and APC-Conjugated Goat Anti-Rabbit IgG(H+L)(red), or 0.25 ug Isotype Control (blue). Cells were fixed with 4% PFA and permeabilized with Flow Cytometry Perm Buffer (PF00011-C). This data was developed using the same antibody clone with 83609-6-PBS in a different storage buffer formulation.

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  Affinity and Kinetic Characterization of 83609-6-RR

  Biolayer interferometry (BLl) kinetic assays of 83609-6-RR against Human Dystrophin were performed. The affinity constant is below 1 pM.

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Product Information

The immunogen of 83609-6-PBS is Dystrophin Fusion Protein expressed in E. coli.

• Tested Reactivity: human, mouse, rat
• Host / Isotype: Rabbit / IgG
• Class: Recombinant
• Type: Antibody
• Immunogen: Dystrophin fusion protein Ag3408
• Full Name: dystrophin
• Calculated Molecular Weight: 3685 aa, 427 kDa
• GenBank Accession Number: BC028720
• Gene Symbol: Dystrophin
• Gene ID (NCBI): 1756
• Conjugate: Unconjugated
• Form: Liquid
• Purification Method: Protein A purfication
• Storage Buffer: PBS Only
• Storage Conditions: Store at -80°C.

Background Information

Dystrophin (DMD or BMD) is a large muscle protein whose mutations cause Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), the childhood neuromuscular disorders that result in progressive muscle weakness, respiratory difficulties and cardiovascular dysfunction. Dystrophin is a crucial component of the dystrophin-glycoprotein complex which is essential for muscle membrane integrity and stability. Dystrophin is located on the cytoplasmic face of the sarcolemma and connects the cytoskeletal network to the sarcolemma and extracellular matrix. Multiple isoforms of dystrophin exist due to the alternative splicing, with a wide range of MW (69-72, 110-143, 271, 426 kDa). Most tissues contain transcripts of several isoforms.