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{{Infobox_gene}}
{{PBB_Controls
'''Glycoprotein Ib (platelet), beta polypeptide''' ('''GP1BB''') also known as '''CD42c''' ('''C'''luster of '''D'''ifferentiation '''42c'''), is a protein that in humans is encoded by the GP1BB gene.<ref name="entrez">{{cite web | title = Entrez Gene: GP1BB glycoprotein Ib (platelet), beta polypeptide| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2812| accessdate = }}</ref>
| update_page = yes
| require_manual_inspection = no
| update_protein_box = yes
| update_summary = yes
| update_citations = yes
}}


<!-- The GNF_Protein_box is automatically maintained by Protein Box Bot.  See Template:PBB_Controls to Stop updates. -->
== Function ==
{{GNF_Protein_box
| image =
| image_source =
| PDB =
| Name = Glycoprotein Ib (platelet), beta polypeptide
| HGNCid = 4440
| Symbol = GP1BB
| AltSymbols =; CD42c
| OMIM = 138720
| ECnumber = 
| Homologene = 30972
| MGIid = 107852
| Function = {{GNF_GO|id=GO:0004888 |text = transmembrane receptor activity}} {{GNF_GO|id=GO:0005515 |text = protein binding}}
| Component = {{GNF_GO|id=GO:0005887 |text = integral to plasma membrane}} {{GNF_GO|id=GO:0016020 |text = membrane}}
| Process = {{GNF_GO|id=GO:0007155 |text = cell adhesion}} {{GNF_GO|id=GO:0007166 |text = cell surface receptor linked signal transduction}} {{GNF_GO|id=GO:0030168 |text = platelet activation}}
| Orthologs = {{GNF_Ortholog_box
    | Hs_EntrezGene = 2812
    | Hs_Ensembl = 
    | Hs_RefseqProtein = NP_000398
    | Hs_RefseqmRNA = NM_000407
    | Hs_GenLoc_db = 
    | Hs_GenLoc_chr = 
    | Hs_GenLoc_start = 
    | Hs_GenLoc_end = 
    | Hs_Uniprot = 
    | Mm_EntrezGene = 14724
    | Mm_Ensembl = ENSMUSG00000050761
    | Mm_RefseqmRNA = NM_001001999
    | Mm_RefseqProtein = NP_001001999
    | Mm_GenLoc_db = 
    | Mm_GenLoc_chr = 16
    | Mm_GenLoc_start = 18534285
    | Mm_GenLoc_end = 18534929
    | Mm_Uniprot = P56400
  }}
}}
'''Glycoprotein Ib (platelet), beta polypeptide''' ('''GP1BB''') also known as '''CD42c''' ('''C'''luster of '''D'''ifferentiation '''42c'''), is a human [[gene]].<ref name="entrez">{{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 = }}</ref>


<!-- The PBB_Summary template is automatically maintained by Protein Box Bot.  See Template:PBB_Controls to Stop updates. -->
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.<ref name="entrez" />
{{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.<ref name="entrez">{{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 = }}</ref>
}}


==See also==
== Interactions ==
 
GP1BB has been shown to [[Protein-protein interaction|interact]] with [[YWHAZ]].<ref name=pmid9454760>{{cite journal | vauthors = Calverley DC, Kavanagh TJ, Roth GJ | title = Human signaling protein 14-3-3zeta interacts with platelet glycoprotein Ib subunits Ibalpha and Ibbeta | journal = Blood | volume = 91 | issue = 4 | pages = 1295–303  | date = February 1998 | pmid = 9454760 }}</ref><ref name=pmid8034572>{{cite journal | vauthors = Du X, Harris SJ, Tetaz TJ, Ginsberg MH, Berndt MC | 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  | date = July 1994 | pmid = 8034572 }}</ref><ref name=pmid10627461>{{cite journal | vauthors = Feng S, Christodoulides N, Reséndiz JC, Berndt MC, Kroll MH | 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  | date = January 2000 | pmid = 10627461 }}</ref>
 
== See also ==
* [[Cluster of differentiation]]
* [[Cluster of differentiation]]


==References==
== References ==
{{reflist|2}}
{{reflist}}


==Further reading==
== Further reading ==
{{refbegin | 2}}
{{refbegin | 2}}
{{PBB_Further_reading
* {{cite journal | vauthors = 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 = 2002 | pmid = 12463594 | doi = 10.1007/BF02982690 }}
| citations =
* {{cite journal | vauthors = 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 = 10.1160/th07-05-0327 }}
*{{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 | vauthors = Wardell MR, Reynolds CC, Berndt MC, Wallace RW, Fox JE | 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=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 | vauthors = Andrews RK, Booth WJ, Gorman JJ, Castaldi PA, Berndt MC | 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 = 1989 | pmid = 2557900 | doi = 10.1021/bi00447a009 }}
*{{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 | vauthors = Berndt MC, Gregory C, Kabral A, Zola H, Fournier D, Castaldi PA | 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 = 10.1111/j.1432-1033.1985.tb09152.x }}
*{{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 | vauthors = Lopez JA, Chung DW, Fujikawa K, Hagen FS, Davie EW, Roth GJ | 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 | pmc = 279943 | doi = 10.1073/pnas.85.7.2135 }}
*{{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 | vauthors = 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 = 10.1016/0006-291X(87)90963-6 }}
*{{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 | vauthors = 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 | pmc = 47349 | doi = 10.1073/pnas.90.18.8327 }}
*{{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 | vauthors = 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 = 10.1074/jbc.270.24.14693 }}
*{{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 | vauthors = 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=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 | vauthors = Du X, Harris SJ, Tetaz TJ, Ginsberg MH, Berndt MC | 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=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 | vauthors = Kelly MD, Essex DW, Shapiro SS, Meloni FJ, Druck T, Huebner K, Konkle BA | 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 | pmc = 294449 | doi = 10.1172/JCI117249 }}
*{{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 | vauthors = Ludlow LB, Schick BP, Budarf ML, Driscoll DA, Zackai EH, Cohen A, Konkle BA | 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 = 10.1074/jbc.271.36.22076 }}
*{{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 | vauthors = 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 | pmc = 507827 | doi = 10.1172/JCI119188 }}
*{{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 | vauthors = Kunishima S, Lopez JA, Kobayashi S, Imai N, Kamiya T, Saito H, Naoe T | 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=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 | vauthors = 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=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 | vauthors = 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 = 10.1046/j.1432-1327.1999.00467.x }}
*{{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 | vauthors = Feng S, Christodoulides N, Reséndiz JC, Berndt MC, Kroll MH | 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=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 | vauthors = Moran N, Morateck PA, Deering A, Ryan M, Montgomery RR, Fitzgerald DJ, Kenny D | 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=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 | vauthors = Kasirer-Friede A, Ware J, Leng L, Marchese P, Ruggeri ZM, Shattil SJ | 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 }}
*{{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 }}
}}
{{refend}}
{{refend}}


==External links==
== External links ==
* {{MeshName|GP1BB+protein,+human}}
* {{MeshName|GP1BB+protein,+human}}


{{membrane-protein-stub}}
{{NLM content}}
{{NLM content}}
{{Clusters of differentiation}}
{{Clusters of differentiation}}
{{Coagulation}}
[[Category:Clusters of differentiation]]
[[Category:Clusters of differentiation]]
{{WikiDoc Sources}}

Latest revision as of 12:24, 9 January 2019

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Identifiers
Aliases
External IDsGeneCards: [1]
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

n/a

n/a

RefSeq (protein)

n/a

n/a

Location (UCSC)n/an/a
PubMed searchn/an/a
Wikidata
View/Edit Human

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.[1]

Interactions

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

See also

References

  1. 1.0 1.1 "Entrez Gene: GP1BB glycoprotein Ib (platelet), beta polypeptide".
  2. Calverley DC, Kavanagh TJ, Roth GJ (February 1998). "Human signaling protein 14-3-3zeta interacts with platelet glycoprotein Ib subunits Ibalpha and Ibbeta". Blood. 91 (4): 1295–303. PMID 9454760.
  3. Du X, Harris SJ, Tetaz TJ, Ginsberg MH, Berndt MC (July 1994). "Association of a phospholipase A2 (14-3-3 protein) with the platelet glycoprotein Ib-IX complex". J. Biol. Chem. 269 (28): 18287–90. PMID 8034572.
  4. Feng S, Christodoulides N, Reséndiz JC, Berndt MC, Kroll MH (January 2000). "Cytoplasmic domains of GpIbalpha and GpIbbeta regulate 14-3-3zeta binding to GpIb/IX/V". Blood. 95 (2): 551–7. PMID 10627461.

Further reading

External links

This article incorporates text from the United States National Library of Medicine, which is in the public domain.