Technical Data
F4195X
Fibrillin 1 (BSA & Azide Free)
Description:
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 Electron Microscopy, Immunofluorescence, Immunohistochemistry and Western Blot. Other applications not tested.

Recommended Dilution:
Immunohistochemistry: Acetone-fixed and frozen sections
Western Blot: 1-2ug/ml for 2hrs RT
Optimal dilutions to be determined by the researcher.

Hybridoma:
Produced by the fusion between BALB/c splenocytes and mouse myeloma P3 X 63A8.653 (NS1) cells.

Positive Control:
Normal skin fibroblasts

Cellular Localization:
Extracellular microfibrils

Storage and Stability:
May be stored at 4C for short-term only. For long-term storage and to avoid repeated freezing and thawing, add sterile glycerol (40-50%), aliquot and store at -20C or colder. Aliquots are stable for at least 12 months at -20C. 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.
TypeIsotypeCloneGrade
MabIgG1,k0.N.294Affinity Purified
SizeStorageShippingSourceHost
100ug-20CBlue IceBovineMouse
Concentration:
1mg/ml
Immunogen:
Microfibrils from zonular apparatus of bovine eye.
Purity:
Purified by Protein G affinity chromatography.
Form
Supplied as a liquid in PBS, pH 7.4. Also available with BSA and azide. See F4195.
Specificity:
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.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
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).