Fibrillin 1

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 frozen sections\nWestern Blot: 1-2ug/ml for 2hrs at RT.\nOptimal dilutions to be determined by the researcher.\n\nPositive Control: \nNormal skin fibroblasts\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 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

Size

500ul

Applications

IF, IHC, WB

Hosts

Mouse

Reactivities

Hum, Bov

Form

Supplied as a liquid in PBS, pH 7.4, 0.2% BSA, 0.09% sodium azide. Also available without BSA and azide, see F4195X.

P Type

Mab

Purity

Purified by Protein G affinity chromatography.

Isotype

IgG1,k

References

1. Maier, A., et al., Investigative Ophthalmology and Visual Science 35(7): 3103-3110 (1994). 2. Wright, D.W., et al., Matrix Biology 14(1): 41-49 (1994).

Additional Info

Recognizes bovine Fibrillin-1, 350kD. 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. Does not recognize Fibrillin 2. \nSpecies Crossreactivity: Human