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Mouse Anti-GP1BA Recombinant Antibody (13H157) (CBMAB-C2894-LY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
13H157
Antibody Isotype
IgG1
Application
FC, IF

Basic Information

Immunogen
Human platelets
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
10mg BSA
Concentration
0.1 mg/ml
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Glycoprotein Ib Platelet Alpha Subunit
Entrez Gene ID
2811
UniProt ID
P07359
Alternative Names
Glycoprotein Ib Platelet Alpha Subunit; Glycoprotein Ib (Platelet), Alpha Polypeptide; Platelet Glycoprotein Ib Alpha Chain; Antigen CD42b-Alpha; GP-Ib Alpha; GPIbA; Platelet Membrane Glycoprotein 1b-Alpha Subunit; Mutant Platelet Membrane Glycoprotein Ib-Alpha; Platelet Membrane Glycoprotein Ib-Alpha; Glycoprotein Ibalpha; CD42b Antigen;
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.
More Infomation

Dib, F., Quéméner, A., Bayart, S., Boisseau, P., Babuty, A., Trossaërt, M., ... & Fouassier, M. (2022). Biological, clinical features and modelling of heterozygous variants of glycoprotein Ib platelet subunit alpha (GP1BA) and glycoprotein Ib platelet subunit beta (GP1BB) genes responsible for constitutional thrombocytopenia. British Journal of Haematology.

Xu, Z. Y., Xu, Y., Xie, X. F., Tian, Y., Sui, J. H., Sun, Y., ... & Fan, Y. J. (2021). Anti-platelet aggregation of Panax notoginseng triol saponins by regulating GP1BA for ischemic stroke therapy. Chinese medicine, 16(1), 1-14.

Leinøe, E., Brøns, N., Rasmussen, A. Ø., Gabrielaite, M., Zaninetti, C., Palankar, R., ... & Rossing, M. (2021). The Copenhagen founder variant GP1BA c. 58T> G is the most frequent cause of inherited thrombocytopenia in Denmark. Journal of Thrombosis and Haemostasis, 19(11), 2884-2892.

Ma, J., Chen, Z., Li, G., Gu, H., & Wu, R. (2021). A novel mutation in GP1BA gene in a family with autosomal dominant Bernard Soulier syndrome variant: A case report. Experimental and Therapeutic Medicine, 21(4), 1-1.

Nagy, Z., Vögtle, T., Geer, M. J., Mori, J., Heising, S., Di Nunzio, G., ... & Senis, Y. A. (2019). The Gp1ba-Cre transgenic mouse: a new model to delineate platelet and leukocyte functions. Blood, The Journal of the American Society of Hematology, 133(4), 331-343.

Gollomp, K., & Poncz, M. (2019). Gp1ba-Cre or Pf4-Cre: pick your poison. Blood, The Journal of the American Society of Hematology, 133(4), 287-288.

Chang, W. A., Sheu, C. C., Liu, K. T., Shen, J. H., Yen, M. C., & Kuo, P. L. (2018). Identification of mutations in SLC4A1, GP1BA and HFE in a family with venous thrombosis of unknown cause by next‑generation sequencing. Experimental and Therapeutic Medicine, 16(5), 4172-4180.

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For research use only. Not intended for any clinical use.

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