|Other Names||Multimerin-1, EMILIN-4, Elastin microfibril interface located protein 4, Elastin microfibril interfacer 4, Endothelial cell multimerin, Platelet glycoprotein Ia*, 155 kDa platelet multimerin, p-155, p155, MMRN1, ECM, EMILIN4, GPIA*, MMRN|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Synonyms||ECM, EMILIN4, GPIA*, MMRN|
|Function||Carrier protein for platelet (but not plasma) factor V/Va. Plays a role in the storage and stabilization of factor V in platelets. Upon release following platelet activation, may limit platelet and plasma factor Va-dependent thrombin generation. Ligand for integrin alpha-IIb/beta-3 and integrin alpha-V/beta-3 on activated platelets, and may function as an extracellular matrix or adhesive protein.|
|Tissue Location||Synthesized by endothelial cells and megakaryocytes. Stored in platelet alpha granules and endothelial cell Weibel-Palade bodies, following activation of these cells, it is released and attached to megakaryocytes, platelets, endothelium and subendothelium of blood vessels. Not found in plasma. Found in vascular tissues such as placenta, lung, and liver|
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Multimerin is a massive, soluble protein found inplatelets and in the endothelium of blood vessels. It is comprisedof subunits linked by interchain disulfide bonds to form large,variably sized homomultimers. Multimerin is a factor V/Va-bindingprotein and may function as a carrier protein for platelet factorV. It may also have functions as an extracellular matrix oradhesive protein. Recently, patients with an unusualautosomal-dominant bleeding disorder (factor V Quebec) were foundto have a deficiency of platelet multimerin.
Davila, S., et al. Genes Immun. 11(3):232-238(2010)Simon-Sanchez, J., et al. Nat. Genet. 41(12):1308-1312(2009)Tasneem, S., et al. J. Thromb. Haemost. 7(4):685-692(2009)Jeimy, S.B., et al. Thromb. Haemost. 100(6):1058-1067(2008)Jeimy, S.B., et al. Thromb. Res. 123(2):352-354(2008)
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