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Dystrophin (DMD) Antibody

Catalogue No: abx001224
Price: £120.00
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DMD Antibody is a Rabbit Polyclonal antibody against DMD. The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. 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. [provided by RefSeq, Jul 2008].

Target DMD
Clonality Polyclonal
Reactivity Mouse
Tested Applications WB
Host Rabbit
Recommended dilutions WB: 1/200 - 1/2000. Optimal dilutions/concentrations should be determined by the end user.
Conjugation Unconjugated
Immunogen Recombinant protein of human DMD.
Isotype IgG
Form Liquid
Purification Affinity purified.
Storage Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
UniProt Primary AC P11532 (UniProt, ExPASy)
UniProt Entry Name DMD_HUMAN
Gene Symbol DMD
GeneID 1756
String 9606.ENSP00000354923
Molecular Weight Calculated MW: 57-72 kDa, 271 kDa, 425-426 kDa
Observed MW: 427 kDa
Buffer PBS, pH 7.3, 0.02% sodium azide, 50% glycerol.
Concentration > 1 mg/ml
Availability Shipped within 5-10 working days.
Note This product is for research use only.
Research Articles on Dystrophin (DMD) Antibody

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