Product Name: Dystrophin antibody (ready-to-use)
Applications: IHC-P
Predicted Target Size:
Positive Controls: Skeletal muscle
Form Supplied: Liquid
Concentration:
Purification: Immunogen affinity purified
Full Name: dystrophin
Background: 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 extracellular matrix.
Synonyms: Dystrophin, DXS269, DXS272, 1756, DXS268, DXS239, DXS270, DXS142, 300376, DXS206, P11532, DXS230, DMD, DXS164, BMD
Cellular Localization: Cell Membrane
CAS NO: 942487-16-3
Product: VU 0364439
Host: Rabbit
Clonality: Polyclonal
Isotype: IgG
Immunogen: A synthetic peptide corresponding to C-terminal of human Dystrophin.
Antigen Species: Human
Species Reactivity: Human, Rat
Conjugation: Unconjugated
Storage Buffer: PBS, pH 7.4 containing 1%BSA and 0.1% sodium azide
Storage Instruction: Keep as concentrated solution and store at 4°C. Do not freeze.
Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.
Specificity:
PubMed ID:http://www.ncbi.nlm.nih.gov/pubmed/25902488?dopt=Abstract
