catalog number :
MBS2502651
products type :
ELISA Kit
products full name :
Human FBN1 (Fibrillin 1) ELISA Kit
products short name :
[FBN1]
other names :
[fibrillin-1; Fibrillin-1; fibrillin-1; fibrillin 15; fibrillin 1]
products gene name :
[FBN1]
other gene names :
[FBN1; FBN1; FBN; SGS; WMS; MASS; MFS1; OCTD; SSKS; WMS2; ACMICD; ECTOL1; GPHYSD2; FBN]
uniprot entry name :
FBN1_HUMAN
specificity :
This kit recognizes natural and some recombinant Human FBN1. No significant crossreactivity or interference between Human FBN1 and analogues was observed.
storage stability :
Store at 4 degree C.
image1 heading :
Typical Testing Data/Standard Curve (for reference only)
other info1 :
Samples: Serum, Plasma, Biological Fluids. Assay Type: Sandwich. Detection Range: 0.313-20ng/mL. Sensitivity: Min: 0.188ng/mL; Max: 20ng/mL
products description :
Intended Uses: This ELISA kit can be applied to the in vitro quantitative determination of Human FBN1 concentrations in serum, plasma and other biological fluids. Principle of the Assay: This ELISA kit uses Sandwich-ELISA as the method. The micro ELISA plate provided in this kit has been precoated with an antibody specific to Human FBN1. Standards or samples are added to appropriate micro ELISA plate wells and combined with the specific antibody. Then a biotinylated detection antibodies specific for Human FBN1 and Avidin-Horseradish Peroxidase (HRP) conjugate are added to each micro plate well successively and incubated. After incubation, free components are washed away. Then the Substrate Reagent is added to each well, only those wells that contain Human FBN1, biotinylated detection antibody and Avidin- HRP conjugate will appear blue in color. The enzyme-substrate reaction will be terminated by adding Stop Solution and appears yellow in color. The optical density (OD) can be measured with spectrophotometry at a wavelength of 450 nm +/- 2 nm. The OD value is proportional to the concentration of Human FBN1. The concentration of Human FBN1 in samples can be calculated by comparing the OD of the samples with the standard curve.
ncbi acc num :
NP_000129.3
ncbi gb acc num :
NM_000138.4
ncbi mol weight :
312,237 Da
ncbi pathways :
Elastic Fibre Formation Pathway (730310); Extracellular Matrix Organization Pathway (576262); Integrin Cell Surface Interactions Pathway (106110)
ncbi summary :
This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome. [provided by RefSeq, Jul 2008]
uniprot summary :
FBN1: a large, extracellular matrix glycoprotein of the fibrillin family that serves as a structural component of 10-12 nm calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1- containing microfibrils provide long-term force bearing structural support in elastic and nonelastic connective tissue throughout the body. May regulate osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Defects in this protein are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome. Interacts with COL16A1. Protein type: Extracellular matrix; Secreted, signal peptide; Secreted. Chromosomal Location of Human Ortholog: 15q21.1. Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space; extracellular region; microfibril; basement membrane. Molecular Function: protein binding; extracellular matrix structural constituent; calcium ion binding. Biological Process: extracellular matrix disassembly; extracellular matrix organization and biogenesis; heart development; metanephros development; skeletal development. Disease: Ectopia Lentis 1, Isolated, Autosomal Dominant; Mass Syndrome; Weill-marchesani Syndrome 2; Acromicric Dysplasia; Stiff Skin Syndrome; Marfan Syndrome; Geleophysic Dysplasia 2
size5 :
10x96-Strip-Wells