Technical Data
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.

Suitable for use in Electron Microscopy, Immunofluorescence, Immunohistochemistry and Western Blot. Other applications not tested.

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

Positive Control:
Normal skin fibroblasts

Storage and Stability:
May be stored at 4C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
MabIgG1,k0.N.294Affinity Purified
500ul-20CBlue IceBovineMouse
Microfibrils from zonular apparatus of bovine eye. Epitope: aa451-909 Cellular Localization: Extracellular microfibrils
Purified by Protein G affinity chromatography.
Supplied as a liquid in PBS, pH 7.4, 0.2% BSA, 0.09% sodium azide. Also available without BSA and azide, see F4195X.
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. Species Crossreactivity: Human
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
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).