Fbn2; fibrillin 2; BC063774; Fib-2; KIAA4226; mKIAA4226; sne; sy; syndatyly ems; Fbn2 antibody; fibrillin 2 antibody; BC063774 antibody; Fib-2 antibody; KIAA4226 antibody; mKIAA4226 antibody; sne antibody; sy antibody; syndatyly ems antibody; Fibrillin 2; Fibrillin 2 (mouse)

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Goat

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul (lot specific)

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-Fbn2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Peptide ELISA (EIA)

Peptide ELISA: Antibody detection limit dilution 1: 2000.
Western Blot: Not yet tested - our routinely used western blotting protocol does not allow detection of proteins as large as the calculated size of 314kDa according to NP_034311.1. Therefore we cannot recommend an optimal concentration and the product is investigative grade.

313,818 Da

fibrillin 2

fibrillin-2

Fibrillin-2

Fbn-2??[Similar Products]

FBN2_MOUSE

FBN2: 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. Defects in FBN2 are the cause of distal arthrogryposis type 9 (DA9). A form of distal arthrogryposis, a disease characterized by congenital joint contractures that mainly involve two or more distal parts of the limbs, in the absence of a primary neurological or muscle disease. DA9 is a connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears. Belongs to the fibrillin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted, signal peptide; Secreted

Cellular Component: extracellular matrix; extracellular region; microfibril; proteinaceous extracellular matrix

Molecular Function: calcium ion binding; extracellular matrix structural constituent; protein binding

Biological Process: embryonic limb morphogenesis; limb morphogenesis; positive regulation of bone mineralization; positive regulation of osteoblast differentiation

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