GP1BB explained

Glycoprotein Ib (platelet), beta polypeptide (GP1BB) also known as CD42c (Cluster of Differentiation 42c), is a protein that in humans is encoded by the GP1BB gene.^[1]

Function

Platelet glycoprotein Ib (GPIb) is a heterodimeric transmembrane protein consisting of a disulfide-linked 140 kD alpha chain and 22 kD beta chain. It is part of the Glycoprotein Ib-IX-V Receptor Complex (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 1.0 kb mRNA expressed in plateletes and megakaryocytes. A 411 amino acid 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 3.5 kb, expressed in nonhematopoietic tissues such as endothelium, brain and heart, was shown to result from inefficient usage of a non-consensus polyA signal within a separate gene (septin 5) located upstream of this gene. In the absence of polyadenylation from its own imperfect site, the septin 5 gene uses the consensus polyA signal of this gene.

Interactions

GP1BB has been shown to interact with YWHAZ.^{[2] [3] [4]}

See also

• Cluster of differentiation

Further reading

• Kunishima S, Kamiya T, Saito H . Genetic abnormalities of Bernard-Soulier syndrome . Int. J. Hematol. . 76 . 4 . 319–27 . 2002 . 12463594 . 10.1007/BF02982690 . 23635810 .
• Clemetson KJ . A short history of platelet glycoprotein Ib complex . Thromb. Haemost. . 98 . 1 . 63–8 . 2007 . 17597992 . 10.1160/th07-05-0327 . free .
• Wardell MR, Reynolds CC, Berndt MC, Wallace RW, Fox JE . Platelet glycoprotein Ib beta is phosphorylated on serine 166 by cyclic AMP-dependent protein kinase . J. Biol. Chem. . 264 . 26 . 15656–61 . 1989 . 10.1016/S0021-9258(19)84882-2 . 2504723 . free .
• Andrews RK, Booth WJ, Gorman JJ, Castaldi PA, Berndt MC . Purification of botrocetin from Bothrops jararaca venom. Analysis of the botrocetin-mediated interaction between von Willebrand factor and the human platelet membrane glycoprotein Ib-IX complex . Biochemistry . 28 . 21 . 8317–26 . 1989 . 2557900 . 10.1021/bi00447a009 .
• Berndt MC, Gregory C, Kabral A, Zola H, Fournier D, Castaldi PA . Purification and preliminary characterization of the glycoprotein Ib complex in the human platelet membrane . Eur. J. Biochem. . 151 . 3 . 637–49 . 1985 . 3161731 . 10.1111/j.1432-1033.1985.tb09152.x .
• Lopez JA, Chung DW, Fujikawa K, Hagen FS, Davie EW, Roth GJ . The alpha and beta chains of human platelet glycoprotein Ib are both transmembrane proteins containing a leucine-rich amino acid sequence . Proc. Natl. Acad. Sci. U.S.A. . 85 . 7 . 2135–9 . 1988 . 3353370 . 279943 . 10.1073/pnas.85.7.2135 . 1988PNAS...85.2135L . free .
• Canfield VA, Ozols J, Nugent D, Roth GJ . Isolation and characterization of the alpha and beta chains of human platelet glycoprotein Ib . Biochem. Biophys. Res. Commun. . 147 . 2 . 526–34 . 1987 . 3632685 . 10.1016/0006-291X(87)90963-6 .
• Hickey MJ, Hagen FS, Yagi M, Roth GJ . Human platelet glycoprotein V: characterization of the polypeptide and the related Ib-V-IX receptor system of adhesive, leucine-rich glycoproteins . Proc. Natl. Acad. Sci. U.S.A. . 90 . 18 . 8327–31 . 1993 . 7690959 . 47349 . 10.1073/pnas.90.18.8327 . 1993PNAS...90.8327H . free .
• Meyer SC, Fox JE . Interaction of platelet glycoprotein V with glycoprotein Ib-IX regulates expression of the glycoproteins and binding of von Willebrand factor to glycoprotein Ib-IX in transfected cells . J. Biol. Chem. . 270 . 24 . 14693–9 . 1995 . 7782333 . 10.1074/jbc.270.24.14693 . free .
• Yagi M, Edelhoff S, Disteche CM, Roth GJ . Structural characterization and chromosomal location of the gene encoding human platelet glycoprotein Ib beta . J. Biol. Chem. . 269 . 26 . 17424–7 . 1994 . 10.1016/S0021-9258(17)32456-0 . 8021244 . free .
• Du X, Harris SJ, Tetaz TJ, Ginsberg MH, Berndt MC . Association of a phospholipase A2 (14-3-3 protein) with the platelet glycoprotein Ib-IX complex . J. Biol. Chem. . 269 . 28 . 18287–90 . 1994 . 10.1016/S0021-9258(17)32301-3 . 8034572 . free .
• Kelly MD, Essex DW, Shapiro SS, Meloni FJ, Druck T, Huebner K, Konkle BA . Complementary DNA cloning of the alternatively expressed endothelial cell glycoprotein Ib beta (GPIb beta) and localization of the GPIb beta gene to chromosome 22 . J. Clin. Invest. . 93 . 6 . 2417–24 . 1994 . 8200976 . 294449 . 10.1172/JCI117249 .
• Ludlow LB, Schick BP, Budarf ML, Driscoll DA, Zackai EH, Cohen A, Konkle BA . Identification of a mutation in a GATA binding site of the platelet glycoprotein Ibbeta promoter resulting in the Bernard-Soulier syndrome . J. Biol. Chem. . 271 . 36 . 22076–80 . 1996 . 8703016 . 10.1074/jbc.271.36.22076 . free .
• Zieger B, Hashimoto Y, Ware J . Alternative expression of platelet glycoprotein Ib(beta) mRNA from an adjacent 5' gene with an imperfect polyadenylation signal sequence . J. Clin. Invest. . 99 . 3 . 520–5 . 1997 . 9022087 . 507827 . 10.1172/JCI119188 .
• Kunishima S, Lopez JA, Kobayashi S, Imai N, Kamiya T, Saito H, Naoe T . Missense mutations of the glycoprotein (GP) Ib beta gene impairing the GPIb alpha/beta disulfide linkage in a family with giant platelet disorder . Blood . 89 . 7 . 2404–12 . 1997 . 10.1182/blood.V89.7.2404 . 9116284 . free .
• Andrews RK, Harris SJ, McNally T, Berndt MC . Binding of purified 14-3-3 zeta signaling protein to discrete amino acid sequences within the cytoplasmic domain of the platelet membrane glycoprotein Ib-IX-V complex . Biochemistry . 37 . 2 . 638–47 . 1998 . 9425086 . 10.1021/bi970893g .
• Longhurst CM, White MM, Wilkinson DA, Jennings LK . A CD9, alphaIIbbeta3, integrin-associated protein, and GPIb/V/IX complex on the surface of human platelets is influenced by alphaIIbbeta3 conformational states . Eur. J. Biochem. . 263 . 1 . 104–11 . 1999 . 10429193 . 10.1046/j.1432-1327.1999.00467.x .
• Feng S, Christodoulides N, Reséndiz JC, Berndt MC, Kroll MH . Cytoplasmic domains of GpIbalpha and GpIbbeta regulate 14-3-3zeta binding to GpIb/IX/V . Blood . 95 . 2 . 551–7 . 2000 . 10627461 . 10.1182/blood.V95.2.551. 77799615 .
• Moran N, Morateck PA, Deering A, Ryan M, Montgomery RR, Fitzgerald DJ, Kenny D . Surface expression of glycoprotein ib alpha is dependent on glycoprotein ib beta: evidence from a novel mutation causing Bernard-Soulier syndrome . Blood . 96 . 2 . 532–9 . 2000 . 10.1182/blood.V96.2.532 . 10887115 .
• Kasirer-Friede A, Ware J, Leng L, Marchese P, Ruggeri ZM, Shattil SJ . Lateral clustering of platelet GP Ib-IX complexes leads to up-regulation of the adhesive function of integrin alpha IIbbeta 3 . J. Biol. Chem. . 277 . 14 . 11949–56 . 2002 . 11812775 . 10.1074/jbc.M108727200 . free .

External links

• • PDBe-KB provides an overview of all the structure information available in the PDB for Human Platelet glycoprotein Ib beta chain (GP1BB)

Notes and References

1. Web site: Entrez Gene: GP1BB glycoprotein Ib (platelet), beta polypeptide.
2. Calverley DC, Kavanagh TJ, Roth GJ . Human signaling protein 14-3-3zeta interacts with platelet glycoprotein Ib subunits Ibalpha and Ibbeta . Blood . 91 . 4 . 1295–303 . February 1998 . 9454760 . 10.1182/blood.V91.4.1295 . free .
3. Du X, Harris SJ, Tetaz TJ, Ginsberg MH, Berndt MC . Association of a phospholipase A2 (14-3-3 protein) with the platelet glycoprotein Ib-IX complex . J. Biol. Chem. . 269 . 28 . 18287–90 . July 1994 . 10.1016/S0021-9258(17)32301-3 . 8034572 . free .
4. Feng S, Christodoulides N, Reséndiz JC, Berndt MC, Kroll MH . Cytoplasmic domains of GpIbalpha and GpIbbeta regulate 14-3-3zeta binding to GpIb/IX/V . Blood . 95 . 2 . 551–7 . January 2000 . 10627461 . 10.1182/blood.V95.2.551 . 77799615 .