83-5981-38　Dystrophin (DMD) 100ul　052098

特徴

• Dystrophin (UniProt P11532) is encoded by the DMD (also known as BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, MRX85) gene (Gene ID 1756) in human.
• Dystrophin is localized to the inner part of the muscle fiber cell membrane (sarcolemma) and plays an important role in stabilizing the muscle fiber against the mechanical forces of muscle contraction by providing a shock-absorbing connection between the cytoskeleton and the extracellular matrix.
• Duchenne muscular dystrophy (DMD) is caused by gene mutations that disrupt the open reading frame (ORF) and prevent the full translation of dystrophin.
• ORF restoration by exon skipping using antisense oligonucleotides is designed to transform the DMD phenotype to that of the milder disorder, Becker muscular dystrophy (BMD), which is typically caused by in-frame dystrophin deletions that allow the production of an internally deleted, but partially functional dystrophin.
• Application:Suitable for use in Immunohistochemistry, Immunofluorescence and Western Blot.
• Other applications not tested.
• Recommended Dilutions:Immunohistochemistry (FFPE): 1:50 detected Dystrophin in human skeletal muscle myocytes using heat-induced epitope retrieval (HIER)Optimal dilutions to be determined by the researcher.
• Storage and Stability:May be stored at 4℃ for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20℃.
• 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.