ELISA/assay

Human Fibulin 5 ELISA Kit

MBS728254

48-Strip-Wells-(Comp

470 USD

ELISA Kit

Human Fibulin 5 ELISA Kit

Fibulin 5

fibulin-5; Fibulin-5; fibulin-5; urine p50 protein; developmental arteries and neural crest EGF-like protein; fibulin 5; Developmental arteries and neural crest EGF-like protein; Dance; Urine p50 protein; UP50

FBLN5

FBLN5; FBLN5; EVEC; UP50; ADCL2; ARMD3; DANCE; ARCL1A; FIBL-5; DANCE; UNQ184/PRO210; FIBL-5; Dance; UP50

FBLN5_HUMAN

Human

Store all reagents at 2-8 degree C.

Assay Type: Competitive or Sandwich. Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Sensitivity: 0.1 ng/mL.

Signal Transduction

For samples: Serum, plasma, cell culture supernatants, body fluid and tissue homogenate . INTENDED USE This FBLN5 ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human FBLN5. This ELISA kit for research use only, not for therapeutic or diagnostic applications! . PRINCIPLE OF THE ASSAY FBLN5 ELISA kit applies the quantitative sandwich enzyme immunoassay technique. The microtiter plate has been pre-coated with a monoclonal antibody specific for FBLN5. Standards or samples are then added to the microtiter plate wells and FBLN5 if present, will bind to the antibody pre-coated wells. In order to quantitatively determine the amount of FBLN5 present in the sample, a standardized preparation of horseradish peroxidase (HRP)-conjugated polyclonal antibody, specific for FBLN5 a readded to each well to “sandwich” the FBLN5 immobilized on the plate. The microtiter plate undergoes incubation, and then the wells are thoroughly washed to remove all unbound components. Next, substrate solutions are added to each well. The enzyme (HRP) and substrate are allowed to react over a short incubation period. Only those wells that contain FBLN5 and enzyme-conjugated antibody will exhibit a change in color. The enzyme-substrate reaction is terminated by addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The FBLN5 concentration in each sample is interpolated from this standard curve.

19743803

NP_006320.2

NM_006329.3

50,180 Da

Elastic Fibre Formation Pathway (730310); Extracellular Matrix Organization Pathway (576262); Molecules Associated With Elastic Fibres Pathway (730311)

The protein encoded by this gene is a secreted, extracellular matrix protein containing an Arg-Gly-Asp (RGD) motif and calcium-binding EGF-like domains. It promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. It is prominently expressed in developing arteries but less so in adult vessels. However, its expression is reinduced in balloon-injured vessels and atherosclerotic lesions, notably in intimal vascular smooth muscle cells and endothelial cells. Therefore, the protein encoded by this gene may play a role in vascular development and remodeling. Defects in this gene are a cause of autosomal dominant cutis laxa, autosomal recessive cutis laxa type I (CL type I), and age-related macular degeneration type 3 (ARMD3). [provided by RefSeq, Jul 2008]

FBLN5: Promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. Could be a vascular ligand for integrin receptors and may play a role in vascular development and remodeling. Defects in FBLN5 are the cause of cutis laxa, autosomal dominant, type 2 (ADCL2). A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. Additional variable clinical features are gastrointestinal diverticula, hernia, and genital prolapse. Rare manifestations are pulmonary artery stenosis, aortic aneurysm, bronchiectasis, and emphysema. Defects in FBLN5 are a cause of cutis laxa, autosomal recessive, type 1A (ARCL1A). A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. The clinical spectrum of autosomal recessive cutis laxa is highly heterogeneous with respect to organ involvement and severity. Type I autosomal recessive cutis laxa is a specific, life-threatening disorder with organ involvement, lung atelectasis and emphysema, diverticula of the gastrointestinal and genitourinary systems, and vascular anomalies. Associated cranial anomalies, late closure of the fontanel, joint laxity, hip dislocation, and inguinal hernia have been observed but are uncommon. Defects in FBLN5 are the cause of age-related macular degeneration type 3 (ARMD3). ARMD is a multifactorial disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Belongs to the fibulin family. Protein type: Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 14q32.1. Cellular Component: extracellular matrix; extracellular space; proteinaceous extracellular matrix; extracellular region. Molecular Function: integrin binding; protein C-terminus binding; protein binding; protein homodimerization activity; calcium ion binding. Biological Process: elastic fiber assembly; extracellular matrix organization and biogenesis; secretion; cell-matrix adhesion; regulation of cell growth. Disease: Cutis Laxa, Autosomal Dominant 2; Macular Degeneration, Age-related, 3; Cutis Laxa, Autosomal Recessive, Type Ia

48-Strip-Wells-(Competitive)

470 USD

48-Strip-Wells-(Sandwich)

470

96-Strip-Wells-(Competitive)

675

96-Strip-Wells-(Sandwich)

675