Dystrophin Antibody (DMD/6270) [DyLight 488] Summary
Immunogen |
Recombinant fragment (around aa1700-2300) of human Dystrophin (exact sequence is proprietary). |
Localization |
Cell surface. Cytoplasm. |
Isotype |
IgG2a Kappa |
Clonality |
Monoclonal |
Host |
Mouse |
Gene |
DMD |
Purity |
Protein A or G purified |
Innovator's Reward |
Test in a species/application not listed above to receive a full credit towards a future purchase. |
Applications/Dilutions
Dilutions |
- Immunocytochemistry/ Immunofluorescence
- Immunohistochemistry-Paraffin
- Western Blot
|
Application Notes |
Optimal dilution of this antibody should be experimentally determined. |
Reactivity Notes
Does not react with mouse, xenopus or chicken
Packaging, Storage & Formulations
Storage |
Store at 4C in the dark. |
Buffer |
50mM Sodium Borate |
Preservative |
0.05% Sodium Azide |
Purity |
Protein A or G purified |
Notes
DyLight (R) is a trademark of Thermo Fisher Scientific Inc. and its subsidiaries.
Alternate Names for Dystrophin Antibody (DMD/6270) [DyLight 488]
Background
Dystrophin is a muscle membrane protein (427 kDa) which is absent, reduced or altered as a result of mutation in Duchenne and Becker muscular dystrophies (DMD/BMD) or its homologue in the mouse.8 Severe DMD is associated with a marked dystrophin deficiency whereas patients with the milder form of BMD show less pronounced abnormalities of protein expression. Because abnormalities in the protein expression occur specifically in patients with these types of muscular dystrophy, dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. Predictions from the sequence suggest a structural protein on the inner face of the membrane, consisting of a 25-repeat, rod-like triple-helical domain separating an N-terminal actin-binding domain from two C-terminal domains, one of which is rich in cysteine.9 The large size of dystrophin and its low abundance (<0.01% of the total muscle protein) are a hindrance to the isolation of intact, native protein for structure/function studies. Monoclonal antibodies against defined regions10 of dystrophin provide a means for studying its structure and function, interactions with other proteins and the nature of the partial gene products produced in some patients carrying deletions in the dystrophin gene. The antibodies are useful in the prenatal or post-abortion diagnosis of muscular dystrophy carriers by immunohistological analyses.11
Limitations
This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are
guaranteed for 1 year from date of receipt.
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