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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against FBN2. It has beenselected for its ability to recognize FBN2 in immunohistochemical staining andwestern blotting.

Affinity Chromatography

Liquid

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

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.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western blotting: 0.2-2ug/mL;1:250-2500
Immunohistochemistry: 5-20ug/mL;1:25-100
Immunocytochemistry: 5-20ug/mL;1:25-100
Optimal working dilutions must be determined by end user.

Western Blot: Sample: Recombinant protein.

DAB staining on fromalin fixed paraffin- embedded breast cancer tissue)

DAB staining on IHC-P; Samples: Human Breast cancer Tissue)

157,690 Da

fibrillin 2

fibrillin-2

Fibrillin-2

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. [provided by RefSeq, Jul 2008]

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; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 5q23.3

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

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

Biological Process: camera-type eye development; embryonic eye morphogenesis; embryonic limb morphogenesis; extracellular matrix disassembly; extracellular matrix organization; positive regulation of bone mineralization; positive regulation of osteoblast differentiation

Disease: Arthrogryposis, Distal, Type 9; Macular Degeneration, Early-onset

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