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.
Applications:Suitable for use in ELISA.
Other applications have not been tested.
Recommended Dilutions:Optimal dilutions to be determined by the researcher.
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size：100ul
Host：goat
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.
Specificity：Recognizes human Fibrillin 1.
Isotype：IgG
Calc Applications Abbrev：E
Calc Crossreactivity：Hu
Immunogen：Synthetic peptide corresponding to C-DASNIEDQSETEAN, from the internal region of human Fibrillin 1 (NP_000129.2). Species Sequence Homology: mouse, rat, canine and bovine
