| Catalogue No. |
M-1833-100 |
| Description |
Dystrophin is a large cytoskeletal protein (426 kDa) found at the inner surface of muscle fibres. Dystrophin is part of the dystrophin glycoprotein complex (DGC), which bridges the inner cytoskeleton (F actin) and the extracellular matrix. The monoclonal antibody clone MANDYS8 was developed in K.E.Davies’ laboratory (see Nguyen Thi Man et al. 1990). This mouse monoclonal specifically recognizes an epitope located on the rod domain of the human dystrophin molecule. The antibody reacts with dystrophin from human, mouse, rat, rabbit, and porcine, but not canine or frog origin. This epitope is present in normal muscle tissue and in nearly all Becker muscular dystrophies. It is absent in cases of Duchenne muscular dystrophies and in the dystrophic mouse (mdx). The epitope recognized by the antibody is sensitive to formalin fixation and paraffin embedding. |
| Batch No. |
See product label |
| Unit size |
100 ug |
| Antigen |
Recombinant human dystrophin encoding amino-acids 816-1749. |
| Antibody Type |
Monoclonal |
| Isotype |
IgG2b |
| Clone |
8H11/MANDYS8 |
| Other Names |
DMD; Dystrophin-4 |
| Accession |
P11532 DMD_HUMAN |
| Produced in |
Mouse |
| Molecular Weight |
Human dystrophin has a predicted length of 3,685 residues and molecular weight of 427 kDa. |
| Purity |
Ascites |
| Applications |
Immunohistochemistry (IHC) and Western Blotting (WB). A dilution of 1:400 - 1:1,600 is recommended for WB. A concentration of 1:400 - 1:6,000 (ABC detection) is recommended to detect dystrophin in fresh frozen, or acetone fixed, 2% PLP fixed sections. Immunolocalization: stains muscle membranes (no staining of Duchenne muscle membrane). Not recommended for paraffin-embedded tissues. Biosensis recommends optimal dilutions/concentrations should be determined by the end user. |
| Specificity |
The specificity of this antibody has been confirmed by WB and IHC against human samples. |
| Species Against |
Human |
| Cross-reactivity |
Mouse, rat, chicken, rabbit. Does not cross-react with dystrophin of canine or Xenopus origin. |
| Form |
Lyophilized from mouse ascites fluid in 1.2% sodium acetate, 2 mg BSA, with 0.01 mg NaN3 as preservative. |
| Reconstitution |
Reconstitute in 1 mL of PBS (pH 7.2 - 7.6) to achieve an antibody concentration of 100 ug/mL. Centrifuge to remove any insoluble material. |
| Storage |
Store lyophilized antibody at 2-8C. After reconstitution divide into aliquots and store at -20C for up to six months. Store at 2-8C short-term (up to 4 weeks). Avoid repetitive freeze/thaw cycles. |
| Expiry Date |
12 months after purchase (lyophilized). |
| General References |
Nguyen Thi Man, A.J. Cartwright, G.E. Morris, D. Love, J.F. Bloomfield and K.E. Davies. Monoclonal antibodies against defined regions of the muscular dystrophy protein, dystrophin. FEBS Letters, 262 (1990) 237-240.
Nguyen Thi Man and G.E. Morris. Use of epitope libraries to identify exon-specific monoclonal antibodies for characterization of altered dystrophins in muscular dystrophy. American J.Human Genetics, 52 (1993) 1057-1066. |
