Rabbit Anti-NBEAL2 Recombinant Antibody (CBWJN-1181) (CBMAB-N1238-WJ)

Name: Rabbit Anti-NBEAL2 Recombinant Antibody (CBWJN-1181)
SKU: CBMAB-N1238-WJ
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Rabbit

Clone

CBWJN-1181

Application

Specificity

Human

Antibody Isotype

IgG

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!]

Concentration

1.09 mg/mL

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

Target

Full Name

neurobeachin-like 2

Introduction

The protein encoded by this gene contains a beige and Chediak-Higashi (BEACH) domain and multiple WD40 domains, and may play a role in megakaryocyte alpha-granule biogenesis. Mutations in this gene are a cause of gray platelet syndrome. [provided by RefSeq, Dec 2011]

Entrez Gene ID

23218

UniProt ID

Q6ZNJ1

Alternative Names

Neurobeachin Like 2; Neurobeachin-Like Protein 2; Neurobeachin-Like 2; KIAA0540; BDPLT4; GPS;

Function

Probably involved in thrombopoiesis. Plays a role in the development or secretion of alpha-granules, that contain several growth factors important for platelet biogenesis.

Biological Process

Platelet formation Source: UniProtKB
Protein localization Source: GO_Central

Cellular Location

Endoplasmic reticulum

Involvement in disease

Gray platelet syndrome (GPS):
A rare platelet disorder characterized by a selective deficiency in the number and contents of platelet alpha-granules. It is associated with mild to moderate bleeding tendency and moderate thrombocytopenia. The platelets are enlarged and have a gray appearance on light microscopy of Wright-stained peripheral blood smears due to decreased granules.

Wegner, P., Drube, J., Ziegler, L., Strotmann, B., Marquardt, R., Küchler, C., ... & Drube, S. (2024). The Neurobeachin‐like 2 protein (NBEAL2) controls the homeostatic level of the ribosomal protein RPS6 in mast cells. Immunology.

Delage, L., Carbone, F., Riller, Q., Zachayus, J. L., Kerbellec, E., Buzy, A., ... & Rieux-Laucat, F. (2023). NBEAL2 deficiency in humans leads to low CTLA-4 expression in activated conventional T cells. Nature Communications, 14(1), 3728.

Kat, M., van Moort, I., Bürgisser, P. E., Kuijpers, T. W., Hofman, M., Favier, M., ... & Bierings, R. (2022). Mutations in NBEAL2 do not impact Weibel-Palade body biogenesis and Von Willebrand factor secretion in Gray Platelet Syndrome Endothelial Colony Forming Cells. bioRxiv, 2022-06.

Tariq, H., Perez Botero, J., Higgins, R. A., & Medina, E. A. (2021). Gray Platelet Syndrome Presenting With Pancytopenia, Splenomegaly, and Bone Marrow Fibrosis: Case Report With a Novel NBEAL2 Mutation. American journal of clinical pathology, 156(2), 253-258.

Pluthero, F. G., & Kahr, W. H. (2021). Gray platelet syndrome: NBEAL2 mutations are associated with pathology beyond megakaryocyte and platelet function defects. Journal of Thrombosis and Haemostasis, 19(2), 318-322.

Wen, Y., Su, H., Wang, W., Ren, F., Jiang, H., Wang, J., ... & Chen, Y. (2021). Prognostic Significance of NBEAL2 in Liver hepatocellular carcinoma.

Lo, R. W., Li, L., Pluthero, F. G., Leung, R., Eto, K., & Kahr, W. H. (2020). The endoplasmic reticulum protein SEC22B interacts with NBEAL2 and is required for megakaryocyte α-granule biogenesis. Blood, The Journal of the American Society of Hematology, 136(6), 715-725.

Jia, S., He, Y., Lu, M., Liao, N., Lei, Y., Lauriane, N., ... & Wei, H. (2019). Identification of novel pathogenic F13A1 mutation and novel NBEAL2 gene missense mutation in a pedigree with hereditary congenital factor XIII deficiency. Gene, 702, 143-147.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Isotype control

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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