Typical Testing Data/Standard Curve (for reference only)
Human FBN1 (Fibrillin 1) CLIA Kit | FBN1 clia kit
Human FBN1 (Fibrillin 1) CLIA Kit
Typical Testing Data/Standard Curve (for reference only)
Typical Testing Data/Standard Curve (for reference only)
Principle of the Assay: This kit uses Sandwich-CLIA as the method. The micro CLIA plate provided in this kit has been pre-coated with an antibody specific to FBN1. Standards or samples are added to the appropriate micro CLIA plate wells and combined with the specific antibody. Then a biotinylated detection antibody specific for FBN1 and Avidin-Horseradish Peroxidase (HRP) conjugate is added to each micro plate well successively and incubated. Free components are washed away. The substrate solution is added to each well. Only those wells that contain FBN1, biotinylated detection antibody and Avidin-HRP conjugate will appear fluorescence. The Relative light unit (RLU) value is measured spectrophotometrically by the Chemiluminescence immunoassay analyzer. The RLU value is positively associated with the concentration of FBN1. You can calculate the concentration of FBN1 in the samples by comparing the RLU value of the samples to the standard curve.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the fibrillin family of proteins. The encoded preproprotein is proteolytically processed to generate a mature extracellular matrix glycoprotein that serves as a structural component of 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 and the related MASS phenotype, as well as ectopia lentis syndrome, Weill-Marchesani syndrome, and Shprintzen-Goldberg syndrome. [provided by RefSeq, Oct 2015]
Uniprot Description
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: Secreted; Secreted, signal peptide; Extracellular matrix
Chromosomal Location of Human Ortholog: 15q21.1
Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space; microfibril; extracellular region; basement membrane
Molecular Function: integrin binding; protein binding; extracellular matrix structural constituent; extracellular matrix constituent conferring elasticity; protein complex binding; calcium ion binding
Biological Process: extracellular matrix disassembly; post-embryonic eye morphogenesis; anatomical structure morphogenesis; extracellular matrix organization and biogenesis; camera-type eye development; embryonic eye morphogenesis; 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
Research Articles on FBN1
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Product Notes
The Human FBN1 fbn1 (Catalog #AAA2532116) is a CLIA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA2532116 CLIA Kit recognizes Human FBN1. It is sometimes possible for the material contained within the vial of "FBN1 (Fibrillin 1), CLIA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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