Product Name: Dystrophin antibody [0.N.258]
Applications: Apuri, EM, ELISA, IHC-Fr, IHC-P
Predicted Target Size:
Positive Controls: Skeletal muscle
Form Supplied: Liquid
Concentration: 0.5 mg/ml (Please refer to the vial label for the specific concentration)
Purification: Protein G 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, CMD3B
Cellular Localization: Cell Membrane
CAS NO: 1001409-50-2
Product: Oxaliplatin
Host: Mouse
Clonality: Monoclonal
Isotype: IgG1
Immunogen: Acetylcholine receptor (AChR) enriched membranes from Torpedo nobiliana electric organ.
Antigen Species: Human
Species Reactivity: Human, Mouse, Rat, Torpedo nobiliana, Xenopus laevis
Conjugation: Unconjugated
Storage Buffer: Preservative: NoneConstituents: 10mM PBS. pH 7.4
Storage Instruction: Keep as concentrated solution. Store at 4ºC short term. For extended storage aliquot and store at -20ºC or below. Avoid freeze-thaw cycles.
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: GTX14452 recognises dystrophin and shows no cross reaction to C protein, a actin, or muscle spectrin.
PubMed ID:http://www.ncbi.nlm.nih.gov/pubmed/25972186?dopt=Abstract
