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Specifications

Clone Host Grade Applications Monoclonal Mouse Affinity Purified B IH IF

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.
Catalog #	F4195X
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 4°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°C or colder. Aliquots are stable for at least 12 months at -20°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.
CAS Number	n/a
Clone Type	Monoclonal
Isotype	IgG1,k
Clone No	0.N.294
Host	Mouse
Source	Bovine
Concentration	1mg/ml
Form	Supplied as a liquid in PBS, pH 7.4. Also available with BSA and azide. See F4195.
Purity	Purified by Protein G affinity chromatography.
Immunogen	Microfibrils from zonular apparatus of bovine eye.
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.
Important Note	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.