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Mouse Anti-GP1BB Recombinant Antibody (CBLG1-1566) (CBMAB-G4453-LY)

This product is antibody recognizes GP1BB. The antibody CBLG1-1566 immunoassay techniques such as: IHC-P, IP, WB.
See all GP1BB antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBLG1-1566
Antibody Isotype
IgG2b
Application
IHC-P, IP, WB

Basic Information

Immunogen
Human platelets
Specificity
Human
Antibody Isotype
IgG2b
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
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 Beta Subunit
Introduction
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 in the neighboring upstream gene (SEPT5, septin 5). In the absence of polyadenylation from its own imperfect site, the SEPT5 gene produces read-through transcripts that use the consensus polyA signal of this gene. [provided by RefSeq, Dec 2010]
Entrez Gene ID
2812
UniProt ID
P13224
Alternative Names
Glycoprotein Ib Platelet Beta Subunit; Glycoprotein Ib (Platelet); Beta Polypeptide; Platelet Glycoprotein Ib Beta Chain; Antigen CD42b-Beta; GP-Ib Beta; GPIBB; Nuclear Localization Signal Deleted In Velocardiofacial Syndrome; Truncated Platelet Membrane Glycoprotein Ib Beta;
Function
Gp-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to von Willebrand factor, which is already bound to the subendothelium.
Biological Process
Cell adhesion Source: UniProtKB-KW
Cell surface receptor signaling pathway Source: ProtInc
Platelet activation Source: UniProtKB
Cellular Location
Membrane
Involvement in disease
Bernard-Soulier syndrome (BSS):
A coagulation disorder characterized by a prolonged bleeding time, unusually large platelets, thrombocytopenia, and impaired prothrombin consumption.
Topology
Extracellular: 27-147
Helical: 148-172
Cytoplasmic: 173-206

Barozzi, S., Pecci, A., Marinoni, M., Fontana, G., Zanchetta, M. E., Noris, P., ... & Faleschini, M. (2023). GP1BB c. 179C> T is the most frequent cause of monoallelic Bernard–Soulier syndrome in the Italian population after the Bolzano variant: a report of two new families. Annals of Hematology, 102(3), 677-679.

Dunstan-Harrison, C., Morison, I. M., & Ledgerwood, E. C. (2022). A novel frameshift GP1BB mutation causes autosomal dominant macrothrombocytopenia with decreased VWF receptor expression but normal platelet aggregation. Platelets, 33(2), 324-327.

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.

Barozzi, S., Bozzi, V., De Rocco, D., Giangregorio, T., Noris, P., Savoia, A., & Pecci, A. (2021). A novel mutation in GP1BB reveals the role of the cytoplasmic domain of GPIbβ in the pathophysiology of Bernard-Soulier syndrome and GPIb-IX complex assembly. International Journal of Molecular Sciences, 22(19), 10190.

Al-Numair, N., Ramzan, K., Alquait, L., Alshehri, M., Imtiaz, F., & Owaidah, T. (2021). A homozygous loss-of-function mutation in GP1BB causing variable clinical phenotypes in a family with Bernard–Soulier syndrome. Blood Coagulation & Fibrinolysis, 32(5), 352-355.

Fiore, M., De Thoré, C., Randrianaivo-Ranjatoelina, H., Baas, M. J., Jacquemont, M. L., Dreyfus, M., ... & Lanza, F. (2020). High prevalence of the natural Asn89Asp mutation in the GP1BB gene associated with Bernard–Soulier Syndrome in French patients from the genetic isolate of Reunion Island. British journal of haematology, 189(3), e67.

Busonero, F., Steri, M., Orrù, V., Sole, G., Olla, S., Marongiu, M., ... & Cucca, F. (2020). A Sardinian founder mutation in glycoprotein Ib platelet subunit beta (GP1BB) that impacts thrombocytopenia. British journal of haematology, 191(5), e124.

Babuty, A., Boisseau, P., Eveillard, M., Ternisien, C., Debord, C., Sigaud, M., ... & Fouassier, M. (2019). A new case of heterozygous variant of the GP1BB gene responsible for macrothrombocytopenia. British journal of haematology, 186(1), 157-159.

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

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