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GP1BA

Glycoprotein Ib (GP Ib) is a platelet surface membrane glycoprotein composed of a heterodimer, an alpha chain and a beta chain, that are linked by disulfide bonds. The Gp Ib functions as a receptor for von Willebrand factor (VWF). The complete receptor complex includes noncovalent association of the alpha and beta subunits with platelet glycoprotein IX and platelet glycoprotein V. The binding of the GP Ib-IX-V complex to VWF facilitates initial platelet adhesion to vascular subendothelium after vascular injury, and also initiates signaling events within the platelet that lead to enhanced platelet activation, thrombosis, and hemostasis. This gene encodes the alpha subunit. Several polymorphisms and mutations have been described in this gene, some of which are the cause of Bernard-Soulier syndromes and platelet-type von Willebrand disease. [provided by RefSeq]
Full Name
Glycoprotein Ib Platelet Alpha Subunit
Function
GP-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to the A1 domain of vWF, which is already bound to the subendothelium.
Biological Process
Blood coagulation Source: MGI
Cell adhesion Source: MGI
Cell morphogenesis Source: Ensembl
Cell surface receptor signaling pathway Source: ProtInc
Fibrinolysis Source: UniProtKB
Platelet activation Source: UniProtKB
Platelet aggregation Source: GO_Central
Regulation of blood coagulation Source: UniProtKB
Cellular Location
Membrane
Involvement in disease
Non-arteritic anterior ischemic optic neuropathy (NAION):
An ocular disease due to ischemic injury to the optic nerve. It usually affects the optic disk and leads to visual loss and optic disk swelling of a pallid nature. Visual loss is usually sudden, or over a few days at most and is usually permanent, with some recovery possibly occurring within the first weeks or months. Patients with small disks having smaller or non-existent cups have an anatomical predisposition for non-arteritic anterior ischemic optic neuropathy. As an ischemic episode evolves, the swelling compromises circulation, with a spiral of ischemia resulting in further neuronal damage.
Bernard-Soulier syndrome (BSS):
A coagulation disorder characterized by a prolonged bleeding time, unusually large platelets, thrombocytopenia, and impaired prothrombin consumption.
Bernard-Soulier syndrome A2, autosomal dominant (BSSA2):
A coagulation disorder characterized by mild to moderate bleeding tendency, thrombocytopenia, and an increased mean platelet volume. Some individuals have no symptoms. Mild bleeding tendencies manifest as epistaxis, gingival bleeding, menorrhagia, easy bruising, or prolonged bleeding after dental surgery.
Pseudo-von Willebrand disease (VWDP):
A bleeding disorder characterized by abnormally enhanced binding of von Willebrand factor by the platelet glycoprotein Ib (GP Ib) receptor complex. Hemostatic function is impaired due to the removal of VWF multimers from the circulation.
Topology
Extracellular: 17-531
Helical: 532-552
Cytoplasmic: 553-652
PTM
Glycocalicin is the product of a proteolytic cleavage/shedding, catalyzed by ADAM17, which releases most of the extracellular domain. Binding sites for vWF and thrombin are in this part of the protein.

Anti-GP1BA antibodies

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Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 6D1
Application*: E
Target: GP1BA
Host: Human
Antibody Isotype: IgG1
Specificity: Human
Clone: CBXC-0730
Application*: F
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBXC-1214
Application*: F
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBYY-C2523
Application*: E, F, WB
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBYY-C0444
Application*: F, IH, WB
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYH-0429
Application*: F
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: MB45
Application*: F
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 5F39
Application*: FC, IF, IH
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 5K236
Application*: FC, IH, WB
Target: GP1BA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBWJC-3737
Application*: WB
Target: GP1BA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBWJC-3736
Application*: WB, P
Target: GP1BA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBWJC-3735
Application*: WB, IP, P
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBWJC-3732
Application*: C, FN
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 8H211
Application*: E, C, WB
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 14D39
Application*: F
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 13H157
Application*: F, IF
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 0.N.114
Application*: IH
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 486805
Application*: WB, F, MC
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBLG1-1559
Application*: IF
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBLG1-1557
Application*: E, F
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBLG1-1556
Application*: F
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: VM16d
Application*: FN, IF
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: TM60
Application*: F
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: SZ2
Application*: C, F
Target: GP1BA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: SP202
Application*: P
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CLB-MB45
Application*: F
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 1C6
Application*: E, IP, WB
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBLG1-181
Application*: F
Target: GP1BA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: HIP1
Application*: F
More Infomation
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
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