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

Polyclonal

IgG

Rabbit

This item recognizes human fibrillin-2, an extracellular matrix protein thought to play a role in the formation of elastic fibers in connective tissue.

Purified
Purified IgG - liquid

IgG concentration 5.0 mg/ml (lot specific)

Store at 4 degree C or at -20 degree C if preferred. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the antibody. Should this product contain a precipitate we recommend microcentrifugation before use.
Shelf Life: 18 months from date of despatch.

Small volumes of anti-FIBRILLIN 2 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.

MBS220842 recognizes human fibrillin-2, an extracellular matrix protein thought to play a role in the formation of elastic fibers in connective tissue.

ELISA (EIA)

157,690 Da

fibrillin 2

fibrillin-2; fibrillin 5

Fibrillin-2

FBN2_HUMAN

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, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 5q23-q31

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

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

Biological Process: positive regulation of osteoblast differentiation; extracellular matrix disassembly; extracellular matrix organization and biogenesis; anatomical structure morphogenesis; positive regulation of bone mineralization; embryonic limb morphogenesis

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

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