GP1BB

Glycoprotein Ib (platelet), beta polypeptide (GP1BB) also known as CD42c (Cluster of Differentiation 42c), is a human gene.cite web | title = Entrez Gene: GP1BB glycoprotein Ib (platelet), beta polypeptide| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2812| accessdate = ]

PBB_Summary
section_title =
summary_text = 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 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.cite web | title = Entrez Gene: GP1BB glycoprotein Ib (platelet), beta polypeptide| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2812| accessdate = ]

ee also

* Cluster of differentiation

References

Further reading

PBB_Further_reading
citations =
*cite journal | author=Kunishima S, Kamiya T, Saito H |title=Genetic abnormalities of Bernard-Soulier syndrome. |journal=Int. J. Hematol. |volume=76 |issue= 4 |pages= 319–27 |year= 2003 |pmid= 12463594 |doi=
*cite journal | author=Clemetson KJ |title=A short history of platelet glycoprotein Ib complex. |journal=Thromb. Haemost. |volume=98 |issue= 1 |pages= 63–8 |year= 2007 |pmid= 17597992 |doi=
*cite journal | author=Wardell MR, Reynolds CC, Berndt MC, "et al." |title=Platelet glycoprotein Ib beta is phosphorylated on serine 166 by cyclic AMP-dependent protein kinase. |journal=J. Biol. Chem. |volume=264 |issue= 26 |pages= 15656–61 |year= 1989 |pmid= 2504723 |doi=
*cite journal | author=Andrews RK, Booth WJ, Gorman JJ, "et al." |title=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. |journal=Biochemistry |volume=28 |issue= 21 |pages= 8317–26 |year= 1990 |pmid= 2557900 |doi=
*cite journal | author=Berndt MC, Gregory C, Kabral A, "et al." |title=Purification and preliminary characterization of the glycoprotein Ib complex in the human platelet membrane. |journal=Eur. J. Biochem. |volume=151 |issue= 3 |pages= 637–49 |year= 1985 |pmid= 3161731 |doi=
*cite journal | author=Lopez JA, Chung DW, Fujikawa K, "et al." |title=The alpha and beta chains of human platelet glycoprotein Ib are both transmembrane proteins containing a leucine-rich amino acid sequence. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=85 |issue= 7 |pages= 2135–9 |year= 1988 |pmid= 3353370 |doi=
*cite journal | author=Canfield VA, Ozols J, Nugent D, Roth GJ |title=Isolation and characterization of the alpha and beta chains of human platelet glycoprotein Ib. |journal=Biochem. Biophys. Res. Commun. |volume=147 |issue= 2 |pages= 526–34 |year= 1987 |pmid= 3632685 |doi=
*cite journal | author=Hickey MJ, Hagen FS, Yagi M, Roth GJ |title=Human platelet glycoprotein V: characterization of the polypeptide and the related Ib-V-IX receptor system of adhesive, leucine-rich glycoproteins. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=90 |issue= 18 |pages= 8327–31 |year= 1993 |pmid= 7690959 |doi=
*cite journal | author=Meyer SC, Fox JE |title=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. |journal=J. Biol. Chem. |volume=270 |issue= 24 |pages= 14693–9 |year= 1995 |pmid= 7782333 |doi=
*cite journal | author=Yagi M, Edelhoff S, Disteche CM, Roth GJ |title=Structural characterization and chromosomal location of the gene encoding human platelet glycoprotein Ib beta. |journal=J. Biol. Chem. |volume=269 |issue= 26 |pages= 17424–7 |year= 1994 |pmid= 8021244 |doi=
*cite journal | author=Du X, Harris SJ, Tetaz TJ, "et al." |title=Association of a phospholipase A2 (14-3-3 protein) with the platelet glycoprotein Ib-IX complex. |journal=J. Biol. Chem. |volume=269 |issue= 28 |pages= 18287–90 |year= 1994 |pmid= 8034572 |doi=
*cite journal | author=Kelly MD, Essex DW, Shapiro SS, "et al." |title=Complementary DNA cloning of the alternatively expressed endothelial cell glycoprotein Ib beta (GPIb beta) and localization of the GPIb beta gene to chromosome 22. |journal=J. Clin. Invest. |volume=93 |issue= 6 |pages= 2417–24 |year= 1994 |pmid= 8200976 |doi=
*cite journal | author=Ludlow LB, Schick BP, Budarf ML, "et al." |title=Identification of a mutation in a GATA binding site of the platelet glycoprotein Ibbeta promoter resulting in the Bernard-Soulier syndrome. |journal=J. Biol. Chem. |volume=271 |issue= 36 |pages= 22076–80 |year= 1996 |pmid= 8703016 |doi=
*cite journal | author=Zieger B, Hashimoto Y, Ware J |title=Alternative expression of platelet glycoprotein Ib(beta) mRNA from an adjacent 5' gene with an imperfect polyadenylation signal sequence. |journal=J. Clin. Invest. |volume=99 |issue= 3 |pages= 520–5 |year= 1997 |pmid= 9022087 |doi=
*cite journal | author=Kunishima S, Lopez JA, Kobayashi S, "et al." |title=Missense mutations of the glycoprotein (GP) Ib beta gene impairing the GPIb alpha/beta disulfide linkage in a family with giant platelet disorder. |journal=Blood |volume=89 |issue= 7 |pages= 2404–12 |year= 1997 |pmid= 9116284 |doi=
*cite journal | author=Andrews RK, Harris SJ, McNally T, Berndt MC |title=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. |journal=Biochemistry |volume=37 |issue= 2 |pages= 638–47 |year= 1998 |pmid= 9425086 |doi= 10.1021/bi970893g
*cite journal | author=Longhurst CM, White MM, Wilkinson DA, Jennings LK |title=A CD9, alphaIIbbeta3, integrin-associated protein, and GPIb/V/IX complex on the surface of human platelets is influenced by alphaIIbbeta3 conformational states. |journal=Eur. J. Biochem. |volume=263 |issue= 1 |pages= 104–11 |year= 1999 |pmid= 10429193 |doi=
*cite journal | author=Feng S, Christodoulides N, Reséndiz JC, "et al." |title=Cytoplasmic domains of GpIbalpha and GpIbbeta regulate 14-3-3zeta binding to GpIb/IX/V. |journal=Blood |volume=95 |issue= 2 |pages= 551–7 |year= 2000 |pmid= 10627461 |doi=
*cite journal | author=Moran N, Morateck PA, Deering A, "et al." |title=Surface expression of glycoprotein ib alpha is dependent on glycoprotein ib beta: evidence from a novel mutation causing Bernard-Soulier syndrome. |journal=Blood |volume=96 |issue= 2 |pages= 532–9 |year= 2000 |pmid= 10887115 |doi=
*cite journal | author=Kasirer-Friede A, Ware J, Leng L, "et al." |title=Lateral clustering of platelet GP Ib-IX complexes leads to up-regulation of the adhesive function of integrin alpha IIbbeta 3. |journal=J. Biol. Chem. |volume=277 |issue= 14 |pages= 11949–56 |year= 2002 |pmid= 11812775 |doi= 10.1074/jbc.M108727200

External links

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