ELISA/assay

Mouse Fibrillin 2 ELISA Kit

MBS729929

48-Strip-Wells

470 USD

ELISA Kit

Mouse Fibrillin 2 ELISA Kit

Fibrillin 2

fibrillin-2; Fibrillin-2; fibrillin-2; fibrillin 5; fibrillin 2

FBN2

FBN2; FBN2; CCA; DA9

FBN2_HUMAN

Mouse

Store all reagents at 2-8 degree C.

Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Assay Type: Competitive. Sensitivity: 1.0 ng/mL.

Cell Biology

Intended Uses: This FBN2 ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Mouse FBN2. This ELISA kit for research use only, not for therapeutic or diagnostic applications!. Principle of the Assay: FBN2 ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-FBN2 antibody and an FBN2-HRP conjugate. The assay sample and buffer are incubated together with FBN2-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the FBN2 concentration since FBN2 from samples and FBN2-HRP conjugate compete for the anti-FBN2 antibody binding site. Since the number of sites is limited, as more sites are occupied by FBN2 from the sample, fewer sites are left to bind FBN2-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The FBN2 concentration in each sample is interpolated from this standard curve.

66346695

NP_001990.2

NM_001999.3

157,690 Da

Elastic Fibre Formation Pathway (730310); Extracellular Matrix Organization Pathway (576262)

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-q31. Cellular Component: extracellular matrix; proteinaceous extracellular matrix; microfibril; extracellular region. 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

48-Strip-Wells

470 USD

96-Strip-Wells

675