Dystrophin Antibody / DMD

• Catalog Number: orb2637283
• Category: Antibodies
• Description: Dystrophin-glycoprotein complex (DGC) connects the F-Actin cytoskeleton on the inner surface of muscle fibers to the surrounding extracellular matrix, through the cell membrane interface. A deficiency in this protein contributes to Duchenne (DMD) and Becker (BMD) muscular dystrophies. The human dystrophin gene measures 2.4 megabases, has more than 80 exons, produces a 14 kb mRNA and contains at least 8 independent tissue-specific promoters and 2 poly A sites. The dystrophin mRNA can undergo differential splicing and produce a range of transcripts that encode a large set of proteins. Dystrophin represents approximately 0.002% of total striated muscle protein and localizes to triadic junctions in skeletal muscle, where it is thought to influence calcium ion homeostasis and force transmission.
• Species/Host: Mouse
• Clonality: Monoclonal
• Clone Number: DMD/3676
• Tested applications: ELISA, IHC-P
• Reactivity: Human
• Isotype: Mouse IgG2b, kappa
• Immunogen: A portion of amino acids 114-263 from the human protein was used as the immunogen for the DMD antibody.
• Antibody Type: Primary Antibody
• Dilution range: ELISA (order BSA-free format for coating),Immunohistochemistry (FFPE): 1-2ug/ml
• Purity: Protein A/G affinity
• Conjugation: Unconjugated
• Formula: 0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced), 0.05% sodium azide
• Hazard Information: This DMD antibody is available for research use only.
• UniProt ID: P11532
• Storage: Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
• Buffer/Preservatives: 0.2 mg/ml in 1X PBS with 0.1 mg/ml rAlbumin (US sourced), 0.05% sodium azide
• Note: For research use only
• Application notes: Optimal dilution of the DMD antibody should be determined by the researcher.
• Expiration Date: 12 months from date of receipt.

IHC staining of FFPE human skeletal muscle tissue with DMD antibody (clone DMD/3676). HIER: boil tissue sections in pH9 10mM Tris with 1mM EDTA for 20 min and allow to cool before testing.

IHC staining of FFPE human heart muscle tissue with DMD antibody (clone DMD/3676). HIER: boil tissue sections in pH9 10mM Tris with 1mM EDTA for 20 min and allow to cool before testing.

SDS-PAGE analysis of purified, BSA-free DMD antibody (clone DMD/3676) as confirmation of integrity and purity.

Analysis of HuProt (TM) microarray containing more than 19000 full-length human proteins using DMD antibody (clone DMD/3676). These results demonstrate the foremost specificity of the DMD/3676 mAb. Z- and S- score: The Z-score represents the strength of a signal that an antibody (in combination with a fluorescently-tagged anti-IgG secondary Ab) produces when binding to a particular protein on the HuProt (TM) array. Z-scores are described in units of standard deviations (SD's) above the mean value of all signals generated on that array. If the targets on the HuProt (TM) are arranged in descending order of the Z-score, the S-score is the difference (also in units of SD's) between the Z-scores. The S-score therefore represents the relative target specificity of an Ab to its intended target.