Dystrophin, a 427kD rod-like cytoskeletal protein, is a member of beta-spectrin/alpha-actinin family. It consists of an N-terminal actin-binding domain, a central rod domain having 24 spectrin like repeats and a C-terminal cysteine-rich domain and dystroglycan-interacting domain responsible for achoring the dystrophin at the sarcolemma. Dystrophin is found at the inner surface of muscle fibres and functions as a flexible linker between the cytoskeleton and the extracellullar matrix. It is a part of Dystrophin-associated-glycoprotein complex (DPC) which is thought as a scaffold connecting the inner surface of muscle fiber to the extracellullar matrix. Mutations in Dystrophin results in 2 fatal X-linked disorders namely Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD).
Applications:
Suitable for use in Immunohistochemistry. Other applications not tested.
Recommended Dilution:
Immunohistochemistry (formalin fixed paraffin embedded): 1:25-1:50
Optimal dilutions to be determined by the researcher.
Positive Control:
Skeletal muscle
Storage and Stability:
May be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
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