Reactivity: | M,R |
Applications: | IHC/IF |
Host Species: | Rabbit |
Clonality: | Polyclonal |
Full Name: | Dystrophin rabbit polyclonal antibody |
Gene Name: | Dystrophin |
Synonyms: | Dmd, CMD3B, BMD |
Immunogen: | Recombinant protein corresponding to Mouse Dystrophin |
Isotype: | IgG |
Purity: | Affinity purification |
Subcellular location: | Cell membrane, Cytoplasm, Cytoskeleton |
Uniprot ID: | P11531, P11530 |
Product Usage Information
Applications | Species | Dilution | Positive Sample |
IHC/IF | Mouse, Rat | 1: 600-1: 1200 | brain, heart, Skeletal muscle |
Background
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.
Images
Immunofluorescence of paraffin embedded mouse brain using DMD (GB112953) at dilution of 1:650 (400x lens) | |
Immunofluorescence of paraffin embedded mouse heart using DMD (GB112953) at dilution of 1:650 (400x lens) | |
Immunofluorescence of paraffin embedded mouse Skeletal muscle using DMD (GB112953) at dilution of 1:650 (400x lens) | |
Immunofluorescence of paraffin embedded rat brain using DMD (GB112953) at dilution of 1:650 (400x lens) | |
Immunofluorescence of paraffin embedded rat heart using DMD (GB112953) at dilution of 1:650 (400x lens) | |
Immunofluorescence of paraffin embedded rat Skeletal muscle using DMD (GB112953) at dilution of 1:650 (400x lens) |
Storage
Storage | Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
Storage Buffer | PBS with 0.02%sodium azide,100 μg/ml BSA and 50% glycerol. |