ANTI-FIBRILLIN

ANTI-FIBRILLIN

Mouse anti-Fibrillin-1   clone 12A5.18    Human/Bovine

(Paraffin Section/WB/Histochemsitry)

cat# RDI-FIBRILLabm       $438.00/100ug

        RDI-FIBRILLabm-1   $688.00/200ug

Presentation: Protein G purified antibody in 10mM PBS with 0.2% BSA and 15mM NaN3. (Bulk available without BSA/Azide upon request) concentration 200ug/ml

Reactivity: human and bovine

Clone: 12A5.18

Isotype: mIgG1k

Immunogen: microfibrils from zonular appartus of bovine eye

Uses: -Western blot: 1-2ug/ml

-histochemistry (frozen and paraffin sections) 2-4ug/ml 60 min RT

Note:no special pretreatment is required for formalin/paraffin sections(do not heat sections in the citrate buffer)

-immunolocalization by Electron Microscopy

-immunofluorescence 5-10ug/ml

Positive Ctrl: Normal skin fibroblasts

Localization: extracellular microfibrils

Background: In western blot, this Mab recognizes a single protein of 350kDa which is identified as fibrillin-1. It shows no cross-reaction with fibrillin-2. Reactivity with deletion mutants indicates that the epitope spans between aa 451-909 of fibillin-2. In immunohistochemical staining of paraformaldehyde-fixed human skin, this antibody decorates the elastic framework and more specifically the oxytalantic network close to the epidermis. Cultures of normal human skin fibroblasts show discrete staining of extracellular fibrils. No corss observed with fibrillin-2.There is an aberrant expression of fibrillin in Marfan Synsdrome

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[3-7]. 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.

Storage: 2-8 deg c