Fibrillin-1 is an extracellular acidic protein with a high cysteine content and an extended thread-like shape with mosaic composition of different types of extracellular modules. Most of the fibrillin molecule is contributed by 47 epidermal growth factor-like (EGF-like) repeats; 43 of them have a consensus sequence for calcium binding (cb). Other modules including 8-cysteine motifs (seven), hybrid motifs (two), and unique domains (three) are interspersed throughout the molecule. Fibrillin-1 contains a proline-rich region close to the N-terminus that is replaced by a glycine-rich region in fibrillin-2. The fibrillins are found throughout the connective tissue as integral components of extended fibrils. There is an aberrant expression of fibrillin-1 in Marfan syndrome.

Applications: |Suitable for use in Western Blot, Immunofluorescence, Immunoprecipitation and FLISA. Other applications have not been tested.

Optimal dilutions to be determined by the researcher.

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 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Note: Applications are based on unconjugated antibody.

Human Fibrillin 1. Epitope: mapping studies identify the binding site to the N-terminal end of the molecule, between aa45-450.

Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight™550.

Purified

Recognizes human Fibrillin 1. Species Crossreactivity: chicken and bovine