84-5431-13　［取扱停止］CD42c 30ul　534396

特徴

• Platelet glycoprotein Ib (GPIb) is a heterodimeric transmembrane protein consisting of a disulfide-linked 140kD alpha chain and 22kD beta chain.
• It is part of the GPIb-V-IX system that constitutes the receptor for von Willebrand factor (VWF), and mediates platelet adhesion in the arterial circulation.
• GPIb alpha chain provides the VWF binding site, and GPIb beta contributes to surface expression of the receptor and participates in transmembrane signaling through phosphorylation of its intracellular domain.
• Mutations in the GPIb beta subunit have been associated with Bernard-Soulier syndrome, velocardiofacial syndrome and giant platelet disorder.
• The 206 amino acid precursor of GPIb beta is synthesized from a 1.
• kb mRNA expressed in plateletes and megakaryocytes.
• A 411aa protein arising from a longer, unspliced transcript in endothelial cells has been described; however, the authenticity of this product has been questioned.
• Yet another less abundant GPIb beta mRNA species of 3.
• kb, expressed in nonhematopoietic tissues such as endothelium, brain and heart, was shown to result from inefficient usage of a non-consensus polyA signal in the neighboring upstream gene (SEPT5, septin 5).
• In the absence of polyadenylation from its own imperfect site, the SEPT5 gene produces read-through transcripts that use the consensus polyA signal of GP1BB.
• Applications:Suitable for use in Immunohistochemistry (paraffin), ELISA.
• Other applications not tested.
• Recommended Dilution:Immunohistochemistry (paraffin): 1:50-1:200ELISA: 1:10,000-1:20,000Optimal dilutions to be determined by the researcher.
• 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.