Technical Data
F4195-03F
Fibrillin 1 (Fibrillin-1, FBN1, FBN, MASS, MFS1, OCTD, SGS, WMS)
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.

Applications:
Suitable for use in ELISA. Other applications have not been tested.

Recommended Dilutions:
Peptide ELISA Titer: 1:2000
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°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.
TypeIsotypeCloneGrade
PabIgGAffinity Purified
SizeStorageShippingSourceHost
100ug-20°CBlue IceHumanGoat
Concentration:
~0.5mg/ml
Immunogen:
Synthetic peptide corresponding to C-DASNIEDQSETEAN, from the Internal region of human Fibrillin 1 (NP_000129.2).
Purity:
Purified by peptide affinity chromatography.
Form
Supplied as a liquid in Tris saline, pH 7.3, 0.5% BSA, 0.02% sodium azide.
Specificity:
Recognizes Fibrillin 1. Species sequence homology: human, mouse, rat, canine, bovine.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
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).
Human mutation 2009 Jul : .