SCD42b/GP1Ba, oplosbaar

CD42b, also known as GP1Ba (GP1B alpha, Glycoprotein 1Ba) is a single pass transmembrane glycoprotein that functions as the key ligand binding subunit of the GP1B platelet surface receptor. The association of CD42b/GP1Ba with GP1Bb (covalently) and platelet glycoproteins IX and V (non‐covalently) forms the von Willebrand factor receptor. The binding of von Willebrand factor (VWF) to its platelet receptor initiates the primary mechanism for the adhesion of platelets to a site of vascular injury and subsequent platelet activation. Additionally, the cytoplasmic (C‐terminal) domain of CD42b/GP1Ba can bind and activate signal transduction molecules, including 14‐3‐3ζ and β‐filamin. Mutations in von Willebrand factor and to a lesser extent, CD42b/GP1Ba, that affect the binding of VWF to the GP1B receptor are the primary cause of the hereditary bleeding disorder known as Type 2 von Willebrand disease (VWD) . Mutations in the CD42b/GP1Ba gene have also been linked to a related bleeding disorder, Bernard‐Soulier disease. Recombinant Human sCD42b/GP1Ba is a 54.6 kDa protein containing 496 amino acid residues that correspond to the extracellular portion of CD42b/GP1Ba, plus a C‐terminal His‐Tag. Due to glycosylation, it migrates at approximately 100‐115 kDa by SDS‐PAGE analysis under reducing and non‐reducing conditions.

Soluble CD42b, antigen CD42b‐alpha, platelet glycoprotein Ib alpha chain, GP‐Ib alpha, glycoprotein Ib platelet alpha subunit, mutant platelet membrane glycoprotein Ib‐alpha

2811

P07359

NP_000164.5

NM_000173.7

17p13.2

Human

Human

HPICEVSKVA SHLEVNCDKR NLTALPPDLP KDTTILHLSE NLLYTFSLAT LMPYTRLTQL NLDRCELTKL QVDGTLPVLG TLDLSHNQLQ SLPLLGQTLP ALTVLDVSFN RLTSLPLGAL RGLGELQELY LKGNELKTLP PGLLTPTPKL EKLSLANNNL TELPAGLLNG LENLDTLLLQ ENSLYTIPKG FFGSHLLPFA FLHGNPWLCN CEILYFRRWL QDNAENVYVW KQGVDVKAMT SNVASVQCDN SDKFPVYKYP GKGCPTLGDE GDTDLYDYYP EEDTEGDKVR ATRTVVKFPT KAHTTPWGLF YSWSTASLDS QMPSSLHPTQ ESTKEQTTFP PRWTPNFTLH MESITFSKTP KSTTEPTPSP TTSEPVPEPA PNMTTLEPTP SPTTPEPTSE PAPSPTTPEP TSEPAPSPTT PEPTSEPAPS PTTPEPTPIP TIATSPTILV SATSLITPKS TFLTTTKPVS LLESTKKTIP ELDQPPKLHH HHHHHH

< 0.1 ng/µg of protein (< 1EU/µg)

≥ 95% by SDS-PAGE gel and HPLC analyses.

Determined by its ability to bind recombinant human von Willebrand Factor-A2 in a functional ELISA.

496

Lyophilized

54.6 kDa

CHO cells