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Mouse Anti-GP1BB Recombinant Antibody (CBYY-C2524) (CBMAB-C3965-YY)

This product is mouse antibody that recognizes GP1BB. The antibody CBYY-C2524 can be used for immunoassay techniques such as: IP, WB
See all GP1BB antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYY-C2524
Antibody Isotype
IgG2b
Application
IP, WB

Basic Information

Immunogen
Human platelets (P13224)
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!]

Buffer
0.5 mg/mL
Preservative
PBS
Concentration
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Glycoprotein Ib Platelet Beta Subunit
Entrez Gene ID
UniProt ID
Alternative Names
Insulin Like Growth Factor 2 Receptor
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.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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