Dystrophin Antibody / DMD

SKU: orb2642803

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.

Images & Validation

• Tested Applications: IHC-P
• Dilution Range: The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.
• Reactivity: Human
• Application Notes:
  Optimal dilution of the Dystrophin antibody should be determined by the researcher.1. The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.

Key Properties

• Antibody Type: Primary Antibody
• Host: Mouse
• Clonality: Monoclonal
• Isotype: Mouse IgG2b, kappa
• Clone No.: DMD/3242
• Immunogen: Amino acids 114-263 from the human protein were used as the immunogen for the Dystrophin antibody.
• Purification: Protein G affinity chromatography
• Conjugation: Unconjugated

Storage & Handling

• 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: Prediluted in 1X PBS, 0.1 mg/ml rAlbumin, 0.05% sodium azide; For IHC use only
• Expiration Date: 12 months from date of receipt.
• Disclaimer: For research use only

IHC staining of FFPE cardiac muscle with Dystrophin antibody (clone DMD/3242). HIER: boil tissue sections in pH9 10mM Tris with 1mM EDTA for 20 min and allow to cool before testing.

Analysis of HuProt (TM) microarray containing more than 19000 full-length human proteins using Dystrophin antibody (clone DMD/3242). These results demonstrate the foremost specificity of the DMD/3242 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.

SDS-PAGE analysis of purified, BSA-free Dystrophin antibody (clone DMD/3242) as confirmation of integrity and purity.

IHC staining of FFPE skeletal muscle with Dystrophin antibody (clone DMD/3242). HIER: boil tissue sections in pH6, 10mM citrate buffer, for 10-20 min and allow to cool before testing.

Quick Database Links

UniProt

P11532