Dystrophin is a member of the spectrin/alpha-actinin family of actin-binding, triple helix rod-containing proteins.
It is absent or greatly reduced in individuals with the X-linked recessive Duchenne’s muscular dystrophy disorder, as well as in mice with the mdx (murine muscular dystrophy) mutation
Dystrophin is the 427kD protein product of the DMB/BMD gene located on the X chromosome at position Xp21.
Western Blot and immuohistochemistry are the two established methods for the detection of abnormalities of dystrophin expression in muscle biopsies.
Dystrophin abnormalities are thought to occur in 100 per cent of patients with DMD/BMD, although genetic abnormalities may only be detected in up to 65 per cent of cases.
Applications:Suitable for use in Immunohistochemistry.
Other applications have not been tested.
Recommended Dilutions:Immunohistochemistry (Formalin/paraffin): 1:200-1:400 for 30 min at RT.
Staining of formalin-fixed tissues requires boiling tissue sections in 10mM citrate buffer, pH 6.0 for 10-20 min followed by cooling at RT for 20 min
Optimal dilutions to be determined by the researcher.
Positive Control:Skeletal muscle
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：250ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified.
Form：Supplied as a liquid in 10mM PBS, pH 7.6, 0.2% BSA, 15mM sodium azide.
Specificity：Recognizes human Dystrophin. Species Crossreactivity: rat.
Isotype：IgG
Calc Applications Abbrev：IHC
Calc Crossreactivity：Hu Rt
Immunogen：Synthetic peptide corresponding to C-terminal of human Dystrophin. Cellular Localization: Cell membrane.
