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Rabbit Anti-FERMT3 Recombinant Antibody (D8I7V) (CBMAB-K0333-LY)

This product is antibody recognizes FERMT3. The antibody D8I7V immunoassay techniques such as: WB, IHC-P, IP, FC.
See all FERMT3 antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
D8I7V
Antibody Isotype
IgG
Application
WB, IHC-P, IP, FC

Basic Information

Specificity
Human, Mouse, Rat
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!]

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
Fermitin Family Member 3
Introduction
Kindlins are a small family of proteins that mediate protein-protein interactions involved in integrin activation and thereby have a role in cell adhesion, migration, differentiation, and proliferation. The protein encoded by this gene has a key role in the regulation of hemostasis and thrombosis. This protein may also help maintain the membrane skeleton of erythrocytes. Mutations in this gene cause the autosomal recessive leukocyte adhesion deficiency syndrome-III (LAD-III). Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Jan 2010]
Entrez Gene ID
Human83706
Mouse108101
Rat309186
UniProt ID
HumanQ86UX7
MouseQ8K1B8
RatB2GVB9
Research Area
Plays a central role in cell adhesion in hematopoietic cells (PubMed:19234463, PubMed:26359933).

Acts by activating the integrin beta-1-3 (ITGB1, ITGB2 and ITGB3) (By similarity).

Required for integrin-mediated platelet adhesion and leukocyte adhesion to endothelial cells (PubMed:19234460).

Required for activation of integrin beta-2 (ITGB2) in polymorphonuclear granulocytes (PMNs) (By similarity).
Biological Process
Cell-matrix adhesion Source: GO_Central
Integrin activation Source: UniProtKB
Integrin-mediated signaling pathway Source: UniProtKB
Leukocyte cell-cell adhesion Source: UniProtKB
Platelet aggregation Source: UniProtKB
Positive regulation of cell migration Source: UniProtKB
Regulation of cell-cell adhesion mediated by integrin Source: UniProtKB
Substrate adhesion-dependent cell spreading Source: UniProtKB
Cellular Location
Podosome. Present in the F-actin surrounding ring structure of podosomes, which are specialized adhesion structures of hematopoietic cells.
Involvement in disease
Leukocyte adhesion deficiency 3 (LAD3):
A disorder characterized by recurrent bacterial infections without pus formation, leukocytosis and major bleeding disorders.

Tang, Y., Nan, N., Gui, C., Zhou, X., Jiang, W., & Zhou, X. (2022). Blockage of PD‐L1 by FERMT3‐mediated Wnt/β‐catenin signalling regulates chemoresistance and immune evasion of colorectal cancer cells. Clinical and Experimental Pharmacology and Physiology, 49(9), 988-997.

Palagano, E., Slatter, M. A., Uva, P., Menale, C., Villa, A., Abinun, M., & Sobacchi, C. (2022). Corrigendum to" Hematopoietic stem cell transplantation corrects osteopetrosis in a child carrying a novel homozygous mutation in the FERMT3 gene"[Bone 97. 2017 Apr: 126-129. Bone, 158, 116345.

Su, X., Chen, J., Lin, X., Chen, X., Zhu, Z., Wu, W., ... & Zeng, Y. (2021). FERMT3 mediates cigarette smoke-induced epithelial–mesenchymal transition through Wnt/β-catenin signaling. Respiratory research, 22(1), 1-15.

Candelaria, G. D. T. P., Antunes, A. D. A., Pastorino, A. C., Dorna, M. D. B., Zanardo, E. A., Dias, A. T., ... & Garanito, M. P. (2021). Novel FERMT3 and PTPRQ Mutations Associated with Leukocyte Adhesion Deficiency-III and Sensorineural Hearing Loss. Journal of Pediatric Genetics.

Manukjan, G., Wiegering, V. A., Reindl, T., Strauß, G., Klopocki, E., Schulze, H., & Andres, O. (2020). Novel variants in FERMT3 and RASGRP2—Genetic linkage in Glanzmann‐like bleeding disorders. Pediatric Blood & Cancer, 67(2), e28078.

Shahid, S., Zaidi, S., Ahmed, S., Siddiqui, S., Abid, A., Malik, S., & Shamsi, T. (2019). A novel nonsense mutation in FERMT3 causes LAD-III in a Pakistani family. Frontiers in Genetics, 10, 360.

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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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