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Gene Review

GP1BB  -  glycoprotein Ib (platelet), beta polypeptide

Homo sapiens

Synonyms: Antigen CD42b-beta, BDPLT1, BS, CD42C, CD42c, ...
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Disease relevance of GP1BB


High impact information on GP1BB


Chemical compound and disease context of GP1BB


Biological context of GP1BB


Anatomical context of GP1BB

  • Both GPIb alpha and GPIb beta are expressed in endothelial cells (EC) [2].
  • The present report describes a monoclonal antibody (RAM.1) that labeled the 26 kDa GPIbbeta subunit on western blots and coprecipitated the three subunits of the GPIb/IX complex from lysates of platelets and transfected CHO and K562 cells [7].
  • The GPIb beta gene has a simple structure, similar to that of other described megakaryocyte/platelet genes, including those of the GPIb-V-IX system [8].
  • Substitution of either Cys5 or Cys7 by Ala failed to show surface expression of GPIb-IX, suggesting that the Cys5- Cys7 disulfide loop in GPIbbeta is essential for the efficient processing and trafficking of GPIb-IX complexes toward the plasma membrane [9].
  • The membrane-proximal GPIbbeta sequence was analogous to a previously reported calmodulin-binding sequence in the leukocyte adhesion receptor, L-selectin [10].

Associations of GP1BB with chemical compounds


Regulatory relationships of GP1BB


Other interactions of GP1BB

  • This occurs through the interaction of GPIX with GPIb beta [11].
  • The model refutes earlier assumptions about cysteine-cysteine interactions in the amino-terminal region of GPIb beta, and predicts a hydrophobic patch the burial of which may contribute to proper conformation of the fully assembled vWF receptor complex [5].
  • The complex is composed of four membrane-spanning glycoproteins (GP): GPIbalpha, GPIbbeta, GPIX, and GPV [16].
  • The HCDCREL-1 gene possesses a non-consensus polyadenylation signal that apparently is not efficiently utilized, resulting in the expression of a readthrough transcript also containing the platelet GPIb beta coding region [17].

Analytical, diagnostic and therapeutic context of GP1BB


  1. Bernard-Soulier syndrome Kagoshima: Ser 444-->stop mutation of glycoprotein (GP) Ib alpha resulting in circulating truncated GPIb alpha and surface expression of GPIb beta and GPIX. Kunishima, S., Miura, H., Fukutani, H., Yoshida, H., Osumi, K., Kobayashi, S., Ohno, R., Naoe, T. Blood (1994) [Pubmed]
  2. Complementary DNA cloning of the alternatively expressed endothelial cell glycoprotein Ib beta (GPIb beta) and localization of the GPIb beta gene to chromosome 22. Kelly, M.D., Essex, D.W., Shapiro, S.S., Meloni, F.J., Druck, T., Huebner, K., Konkle, B.A. J. Clin. Invest. (1994) [Pubmed]
  3. Role of the intracellular domains of GPIb in controlling the adhesive properties of the platelet GPIb/V/IX complex. Perrault, C., Mangin, P., Santer, M., Baas, M.J., Moog, S., Cranmer, S.L., Pikovski, I., Williamson, D., Jackson, S.P., Cazenave, J.P., Lanza, F. Blood (2003) [Pubmed]
  4. Cyclic AMP-dependent phosphorylation of glycoprotein Ib inhibits collagen-induced polymerization of actin in platelets. Fox, J.E., Berndt, M.C. J. Biol. Chem. (1989) [Pubmed]
  5. Mutation in the leucine-rich repeat C-flanking region of platelet glycoprotein Ib beta impairs assembly of von Willebrand factor receptor. Tang, J., Stern-Nezer, S., Liu, P.C., Matyakhina, L., Riordan, M., Luban, N.L., Steinbach, P.J., Kaler, S.G. Thromb. Haemost. (2004) [Pubmed]
  6. Novel heterozygous missense mutation in the platelet glycoprotein Ib beta gene associated with isolated giant platelet disorder. Kunishima, S., Naoe, T., Kamiya, T., Saito, H. Am. J. Hematol. (2001) [Pubmed]
  7. A novel monoclonal antibody against the extracellular domain of GPIbbeta modulates vWF mediated platelet adhesion. Perrault, C., Moog, S., Rubinstein, E., Santer, M., Baas, M.J., de la Salle, C., Ravanat, C., Dambach, J., Freund, M., Santoso, S., Cazenave, J.P., Lanza, F. Thromb. Haemost. (2001) [Pubmed]
  8. Structural characterization and chromosomal location of the gene encoding human platelet glycoprotein Ib beta. Yagi, M., Edelhoff, S., Disteche, C.M., Roth, G.J. J. Biol. Chem. (1994) [Pubmed]
  9. Disruption of the Cys5-Cys7 disulfide bridge in the platelet glycoprotein Ibbeta prevents the normal maturation and surface exposure of GPIb-IX complexes. González-Manchón, C., Butta, N., Iruín, G., Alonso, S., Ayuso, M.S., Parrilla, R. Thromb. Haemost. (2003) [Pubmed]
  10. Interaction of calmodulin with the cytoplasmic domain of the platelet membrane glycoprotein Ib-IX-V complex. Andrews, R.K., Munday, A.D., Mitchell, C.A., Berndt, M.C. Blood (2001) [Pubmed]
  11. The cysteine knot of platelet glycoprotein Ib beta (GPIb beta) is critical for the interaction of GPIb beta with GPIX. Kenny, D., Morateck, P.A., Montgomery, R.R. Blood (2002) [Pubmed]
  12. Double heterozygosity for mutations in the platelet glycoprotein IX gene in three siblings with Bernard-Soulier syndrome. Wright, S.D., Michaelides, K., Johnson, D.J., West, N.C., Tuddenham, E.G. Blood (1993) [Pubmed]
  13. Regulation of glycoprotein Ib-IX-von Willebrand factor interaction by cAMP-dependent protein kinase-mediated phosphorylation at Ser 166 of glycoprotein Ib(beta). Bodnar, R.J., Xi, X., Li, Z., Berndt, M.C., Du, X. J. Biol. Chem. (2002) [Pubmed]
  14. Glycoprotein (GP) Ib-IX-transfected cells roll on a von Willebrand factor matrix under flow. Importance of the GPib/actin-binding protein (ABP-280) interaction in maintaining adhesion under high shear. Cranmer, S.L., Ulsemer, P., Cooke, B.M., Salem, H.H., de la Salle, C., Lanza, F., Jackson, S.P. J. Biol. Chem. (1999) [Pubmed]
  15. Mutation in the leucine-rich repeat of platelet glycoprotein Ib alpha results in defects in its interaction with immobilized von Willebrand factor under flow. Dong, J., Li, C., Schade, A.J., Fredrickson, B.J., Sun, L., McIntire, L.V., López, J.A. Chin. Med. J. (2000) [Pubmed]
  16. Naturally occurring mutations in glycoprotein Ibalpha that result in defective ligand binding and synthesis of a truncated protein. Kenny, D., Jónsson, O.G., Morateck, P.A., Montgomery, R.R. Blood (1998) [Pubmed]
  17. Structure and expression of the human septin gene HCDCREL-1. Yagi, M., Zieger, B., Roth, G.J., Ware, J. Gene (1998) [Pubmed]
  18. A missense mutation (Tyr88 to Cys) in the platelet membrane glycoprotein Ibbeta gene affects GPIb/IX complex expression--Bernard-Soulier syndrome in the homozygous form and giant platelets in the heterozygous form. Kurokawa, Y., Ishida, F., Kamijo, T., Kunishima, S., Kenny, D., Kitano, K., Koike, K. Thromb. Haemost. (2001) [Pubmed]
  19. A point mutation in glycoprotein IX coding sequence (Cys73 (TGT) to Tyr(TAT)) causes impaired surface expression of GPIb/IX/V complex in two families with Bernard-Soulier syndrome. Noda, M., Fujimura, K., Takafuta, T., Shimomura, T., Fujii, T., Katsutani, S., Fujimoto, T., Kuramoto, A., Yamazaki, T., Mochizuki, T., Matsuzaki, M., Sano, M. Thromb. Haemost. (1996) [Pubmed]
  20. Expression of platelet glycoprotein (GP) V in heterologous cells and evidence for its association with GP Ib alpha in forming a GP Ib-IX-V complex on the cell surface. Li, C.Q., Dong, J.F., Lanza, F., Sanan, D.A., Sae-Tung, G., López, J.A. J. Biol. Chem. (1995) [Pubmed]
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