Fibrillin 1 (BSA & Azide Free)

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.\n\nApplications: \nSuitable for use in Electron Microscopy, Immunofluorescence, Immunohistochemistry and Western Blot. Other applications not tested.\n\nRecommended Dilution:\nImmunohistochemistry: Acetone-fixed and frozen sections\nWestern Blot: 1-2ug/ml for 2hrs RT\nOptimal dilutions to be determined by the researcher.\n\nHybridoma: \nProduced by the fusion between BALB/c splenocytes and mouse myeloma P3 X 63A8.653 (NS1) cells.\n\nPositive Control: \nNormal skin fibroblasts\n\nCellular Localization: \nExtracellular microfibrils\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, add sterile glycerol (40-50%), aliquot and store at -20 degrees C or colder. Aliquots are stable for at least 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

F4195X

Size

100ug

Applications

IF, IHC, WB

Hosts

Mouse

Reactivities

Hum, Bov

Form

Supplied as a liquid in PBS, pH 7.4. Also available with BSA and azide. See F4195.

P Type

Mab

Purity

Purified by Protein G affinity chromatography.

Isotype

IgG1,k

References

1. Maier, A., McDaniels, C.N., Mayne, R., Fibrillin and elastin networks in extrafusal tissue and muscle spindles of bovine extraocular muscles. Investigative Ophthalmology and Visual Science 35(7): 3103-10 (1994). 2. Wright, D.W., McDaniels, C.N., Swasdison, S., Accavitti, M.A., Mayne, P.M., Mayne, R., Immunization with undenatured bovine zonular fibrils results in monoclonal antibodies to fibrillin. Matrix Biology 14(1): 41-9 (1994).

Additional Info

Recognizes Fibrillin-1 (350kD). Reactivity with deletion mutants indicates that the epitope spans between aa451-909 of fibrillin-1. In Immunohistochemistry staining of human skin, decorates elastic framework. More specifically the oxytalantic network close to the epidermis. Cultures of normal human skin fibroblasts show discrete staining of extracellular fibrils. Species Crossreactivity: Bovine, human.