Dystrophin (DMD) Antibody

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	Dystrophin (DMD)
Clonality	Polyclonal
Reactivity	Human, Mouse, Rat
Tested Applications	WB, IHC, IF/ICC
Host	Rabbit
Recommended dilutions	WB: 1/500 - 1/1000, IHC-P: 1/50 - 1/200, IF/ICC: 1/50 - 1/200. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user.
Conjugation	Unconjugated
Immunogen	Recombinant fusion protein containing a sequence corresponding to amino acids 346-635 of human Dystrophin.
Isotype	IgG
Form	Liquid
Purification	Purified by affinity chromatography.
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
NCBI Accession	NP_003997.2
KEGG	hsa:1756
String	9606.ENSP00000354923
Molecular Weight	Calculated MW: 427 kDa Observed MW: 427 kDa
Buffer	PBS, pH 7.3, containing 0.05% Proclin-300, 50% glycerol.
Concentration	> 0.2 mg/ml
Availability	Shipped within 5-10 working days.
Note	This product is for research use only.

Research Articles on Dystrophin (DMD)

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