Dystrophin (DMD) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Recommend 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
Reactivity	Human, Mouse
Host	Rabbit
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 Condition	Store at -20°C as received.
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

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