General description
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)
Immunogen
FBN1 (NP_000129, 2772 a.a. ~ 2871 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
SNKVRILELLPALTTLTNHNRYLIESGNEDGFFKINQKEGISYLHFTKKKPVAGTYSLQISSTPLYKKKELNQLEDKYDKDYLSGELGDNLKMKIQVLLH
Physical form
Solution in phosphate buffered saline, pH 7.4
Legal Information
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Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
| biological source | mouse |
| Quality Segment | 100 |
| conjugate | unconjugated |
| antibody form | purified immunoglobulin |
| antibody product type | primary antibodies |
| clone | 3H6, monoclonal |
| form | buffered aqueous solution |
| species reactivity | human |
| technique(s) | indirect ELISA: suitable,western blot: 1-5 μg/mL |
| isotype | IgG2aκ |
| GenBank accession no. | NM_000138 |
| UniProt accession no. | P35555 |
| shipped in | dry ice |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| Gene Information | human ... FBN1(2200) |
