Fibrillin 1 (Fibrillin-1, FBN1, FBN, MASS, MFS1, OCTD, SGS, WMS)

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. \n\nApplications:\nSuitable for use in ELISA. Other applications have not been tested. \n\nRecommended Dilutions: \nPeptide ELISA Titer: 1:2000\nOptimal dilutions to be determined by the researcher. \n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

F4195-03F

Size

100ug

Applications

ELISA

Hosts

Goat

Reactivities

Hum

Form

Supplied as a liquid in Tris saline, pH 7.3, 0.5% BSA, 0.02% sodium azide.

P Type

Pab

Purity

Purified by peptide affinity chromatography.

Isotype

IgG

References

1. Morisaki H, Akutsu K, Ogino H, Kondo N, Yamanaka I, Tsutsumi Y, Yoshimuta T, Okajima T, Matsuda H, Minatoya K, Sasaki H, Tanaka H, Ishibashi-Ueda H, Morisaki T, Mutation of ACTA2 gene as an important cause of familial and nonfamilial nonsyndromatic thoracic aortic aneurysm and/or dissection (TAAD).\nHuman mutation 2009 Jul : .

Additional Info

Recognizes Fibrillin 1. Species sequence homology: human, mouse, rat, canine, bovine.

Alternative Names

fibrillin 15, WMS, SGS, OCTD, MFS1, MASS, FBN, fibrillin 1, FBN1