FBN1 (Fibrillin 1, FBN, MASS, MFS1, OCTD, SGS, WMS, Marfan Syndrome)
Product Sizes
200ul
£479.00
363073-200UL
About this Product
- SKU:
- 363073
- Application:
- Western Blot, Immunohistochemistry
- Clonality:
- Polyclonal
- Extra Details:
- Fibrillin-1 is a major component of the microfibrils that form a sheath surrounding the amorphous elastin. It is believed that the microfibrils are composed of end-to-end polymers of fibrillin. To date, 3 forms of fibrillin have been described. The fibrillin-1 protein was isolated by Engvall in 1986, and mutations in the gene have been linked to the Marfan syndrome. At present more than 100 different mutations have been described. Trask et al. (1999) found that human FIB1 and FIB2 homodimerized via an N-terminal region and that the interaction was stabilized by disulphide bonds. Dimer formation occurred intracellularly, suggesting that the process of fibrillin aggregation initiates early after biosynthesis of the molecules. Applications: Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry. Other applications not tested. Recommended Dilution: ELISA: 1:100-1:5000 Western Blot: 1:50-400 Immunohistochemistry (frozen): 1:50-500 Immunohistochemistry (paraffin): 1:10-100 Immunocytochemistry: 1:50-500 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Host:
- Rabbit
- Immunogen:
- Recombinant protein corresponding to aa723-902 of human FBN1, expressed in E. coli.
- Isotype:
- IgG
- Physical State:
- Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
- Purity:
- Purified by immunoaffinity chromatography.
- Shipping Conditions:
- Blue Ice
- Specificity:
- Recognizes human FBN1.
- Source:
- human
- Storage Conditions:
- -20[o]C
- Supplier:
- United States Biological
- Type:
- Antibodies:Polyclonal Antibody
