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

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

Mouse

Clone

CBXF-0375

Application

ELISA, WB

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.

More Infomation

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

2201

UniProt ID

P35556

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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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

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Isotype control

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

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