ELISA/assay

Fibulin 5 (FBLN5) ELISA Kit

MBS2024100

48-Strip-Wells

565 USD

ELISA Kit

Fibulin 5 (FBLN5) ELISA Kit

Fibulin 5 (FBLN5)

ARMD3; DANCE; EVEC; UP50; FIBL-5; Dance; Developmental arteries and neural crest EGF-like protein

fibulin-5; Fibulin-5; fibulin-5; 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; FIBL-5; Dance; UP50

FBLN5_HUMAN

Human

448

This assay has high sensitivity and excellent specificity for detection of Fibulin 5 (FBLN5). No significant cross-reactivity or interference between Fibulin 5 (FBLN5) and analogues was observed.

For unopened kit: All the reagents should be kept according to the labels on vials. The Standard, Detection Reagent A, Detection Reagent B and the 96-well strip plate should be stored at -20 degree C upon receipt while the others should be at 4 degree C. For opened kit: When the kit is opened, the remaining reagents still need to be stored according to the above storage condition. Besides, please return the unused wells to the foil pouch containing the desiccant pack, and reseal along entire edge of zip-seal. The stability of kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition. To minimize extra influence on the performance, operation procedures and lab conditions, especially room temperature, air humidity, incubator temperature should be strictly controlled. It is also strongly suggested that the whole assay is performed by the same operator from the beginning to the end.

Samples: Serum, plasma, urine and other biological fluids. Assay Type: Sandwich. Detection Range: 15.63-1000pg/mL. Sensitivity: Typically less than 6.1pg/mL.

Precision: Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level Fibulin 5 (FBLN5) were tested 20 times on one plate, respectively. Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level Fibulin 5 (FBLN5) were tested on 3 different plates, 8 replicates in each plate. CV(%) = SD/meanX100 . Intra-Assay: CV<10% . Inter-Assay: CV<12% . Assay Procedure Summary: 1. Prepare all reagents, samples and standards;. 2. Add 100uL standard or sample to each well. Incubate 2 hours at 37 degree C;. 3. Aspirate and add 100uL prepared Detection Reagent A. Incubate 1 hour at 37 degree C;. 4. Aspirate and wash 3 times;. 5. Add 100uL prepared Detection Reagent B. Incubate 30 minutes at 37 degree C;. 6. Aspirate and wash 5 times;. 7. Add 90uL Substrate Solution. Incubate 15-25 minutes at 37 degree C;. 8. Add 50uL Stop Solution. Read at 450nm immediately.

Principle of the Assay: The test principle applied in this kit is Sandwich enzyme immunoassay. The microtiter plate provided in this kit has been pre-coated with an antibody specific to Fibulin 5 (FBLN5). Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody specific to Fibulin 5 (FBLN5). Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain Fibulin 5 (FBLN5), biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm 10nm. The concentration of Fibulin 5 (FBLN5) in the samples is then determined by comparing the O.D. of the samples to the standard curve.

19743803

NP_006320.2

NM_006329.3

Q9UBX5

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; secretion; extracellular matrix organization and biogenesis; 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

565 USD

96-Strip-Wells

765

5x96-Strip-Wells

2975

10x96-Strip-Wells

5340