Dystrophin (DMD) Rabbit Polyclonal Antibody

CAT#: TA354934

Rabbit anti Dystrophin Polyclonal Antibody

Product Images

WB: The tissue lysate derived from mouse skeletal muscle was immunoblotted by Rabbit anti-Dystrophin at 1:500. Multiple bands between 71kDa-250 kDa were observed.

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  IHC: Mouse skeletal muscle stained with Rabbit anti-Dystrophin antibody, at 1:200 for 10 min at RT. Staining of formalinfixed tissue requires boiling tissue sections in 10 mM Citrate Buffer, pH 6.0 for 10 min followed by cooling at RT for 20 min.

Specifications

Product Data

• Applications: IHC, WB
• Recommended Dilution: WB 0.1-1 µg/ml ELISA 0.01-0.1 µg/ml IP 2-5 µg/ml IHC 2-10 µg/ml FC 5-10 µg/ml
• Reactivities: Human, Mouse
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: A synthetic peptide corresponding to the intra domain 410-450aa of human Dystrophin protein. This sequence is identical to mouse and Pan troglodytes and other species.
• Formulation: This affinity purified antibody is supplied in sterile Phosphate buffered saline (pH7.2) containing antibody stabilizer.
• Purification: The Rabbit IgG is purified by Epitope Affinity Purification
• Conjugation: Unconjugated
• Storage: Store at -20°C as received.
• Stability: Stable for 12 months from date of receipt.
• Predicted Protein Size: >110 kDa
• Gene Name: dystrophin
• Database Link:
  NP_000100
  Entrez Gene 13405 MouseEntrez Gene 1756 Human
  UniProt ID P11532
• Background: The dystrophin gene is the largest gene found in nature. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. IHC staining of normal muscle tissue results in clear labeling confined to the periphery (plasma membrane) of normal muscle fibers. The product exhibits wide interspecies cross-reactivity.
• Synonyms: BMD; CMD3B; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; MRX85

Reference Data

• Protein Pathways: Arrhythmogenic right ventricular cardiomyopathy (ARVC), Dilated cardiomyopathy, Hypertrophic cardiomyopathy (HCM), Viral myocarditis