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Mouse Anti-FBN2 Recombinant Antibody (CBXF-0375) (CBMAB-F3144-CQ)

This product is a mouse antibody that recognizes FBN2. The antibody CBXF-0375 can be used for immunoassay techniques such as: S-ELISA, WB, ELISA.
See all FBN2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXF-0375
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Immunogen
FBN2 (NP_001990.2, 2776 a.a.-2876 a.a) partial recombinant protein with GST tag
Specificity
Human
Antibody Isotype
IgG2a, κ
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
PBS, pH 7.4
Concentration
0.5 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.

Target

Full Name
fibrillin 2
Introduction
The protein encoded by this gene is a component of connective tissue microfibrils and may be involved in elastic fiber assembly. Mutations in this gene cause congenital contractural arachnodactyly.
Entrez Gene ID
UniProt ID
Alternative Names
Fibrillin 2; Fibrillin 5; Congenital Contractural Arachnodactyly; Fibrillin-2; EOMD; DA9; CCA;
Research Area
Fibrillin-2:
Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2-containing microfibrils regulate the early process of elastic fiber assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.

Placensin:
Hormone secreted by trophoblasts that promotes trophoblast invasiveness (PubMed:32329225).

Has glucogenic activity: is able to increase plasma glucose levels (By similarity).
Biological Process
Anatomical structure morphogenesis Source: GO_Central
Bone trabecula formation Source: BHF-UCL
Camera-type eye development Source: UniProtKB
Embryonic eye morphogenesis Source: UniProtKB
Embryonic limb morphogenesis Source: Ensembl
Positive regulation of bone mineralization Source: BHF-UCL
Positive regulation of osteoblast differentiation Source: BHF-UCL
Sequestering of TGFbeta in extracellular matrix Source: BHF-UCL
Cellular Location
Secreted. Fibrillin-2 and Placensin chains are still linked together during the secretion from cells, but are subsequently separated by furin.
Fibrillin-2: Extracellular matrix
Placensin: Secreted. Secreted by placental cells.
Involvement in disease
Contractural arachnodactyly, congenital (CCA):
An autosomal dominant connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears.
Macular degeneration, early-onset (EOMD):
An ocular disorder characterized by macular changes resulting in progressive loss of visual acuity.
PTM
Placensin: N-glycosylated.

Xu, F., Guo, D., Jiang, Q., Zhang, R., Yu, T., Yin, X., ... & Bi, H. (2022). Association between anti‐fibrillin‐2 protein induced retinal degeneration via intravitreous delivery and activated TGF‐β signalling in mice. Clinical and Experimental Pharmacology and Physiology, 49(5), 586-595.

Mead, T. J., Martin, D. R., Wang, L. W., Cain, S. A., Gulec, C., Cahill, E., ... & Apte, S. S. (2022). Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development. Elife, 11, e71142.

Peeters, S., Decramer, A., Cain, S. A., Houpt, P., Verstreken, F., Noyez, J., ... & Mortier, G. (2021). Delineation of a new fibrillin-2-opathy with evidence for a role of FBN2 in the pathogenesis of carpal tunnel syndrome. Journal of Medical Genetics, 58(11), 778-782.

Williamson, D. B., Sohn, C. J., Ito, A., & Haltiwanger, R. S. (2021). POGLUT2 and POGLUT3 O-glucosylate multiple EGF repeats in fibrillin-1,-2, and LTBP1 and promote secretion of fibrillin-1. Journal of Biological Chemistry, 297(3).

Hong, Q., Li, R., Zhang, Y., & Gu, K. (2020). Fibrillin 2 gene knockdown inhibits invasion and migration of lung cancer cells. Cellular and Molecular Biology, 66(7), 190-196.

van Loon, K., Yemelyanenko-Lyalenko, J., Margadant, C., Griffioen, A. W., & Huijbers, E. J. (2020). Role of fibrillin-2 in the control of TGF-β activation in tumor angiogenesis and connective tissue disorders. Biochimica et Biophysica Acta (BBA)-Reviews on Cancer, 1873(2), 188354.

Holopainen, S. (2019). The genetics of canine hip dysplasia: the role of fibrillin 2 variant in four dog breeds.

Xu, F. R., Jiang, W. J., Zhang, T., Jiang, Q., Zhang, R. X., & Bi, H. S. (2019). Fibrillin-2 gene mutations associated with hereditary connective tissue diseases. Yi Chuan= Hereditas, 41(10), 919-927.

Yin, W., Kim, H. T., Wang, S., Gunawan, F., Li, R., Buettner, C., ... & Stainier, D. Y. (2019). Fibrillin-2 is a key mediator of smooth muscle extracellular matrix homeostasis during mouse tracheal tubulogenesis. European Respiratory Journal, 53(3).

Lee, Y. J., Park, S. Y., Park, E. K., & Kim, J. E. (2019). Unique cartilage matrix-associated protein regulates fibrillin-2 expression and directly interacts with fibrillin-2 protein independent of calcium binding. Biochemical and biophysical research communications, 511(2), 221-227.

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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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