Technical Data
F4195-11C
Fibrillin 2 (Fibrillin-2, FBN2, Congenital Contractural Arachnodactyly, CCA, DA9)
Description:
Fibrillin-2 is a part of the Fibrillin family of glycoproteins, which are essential factors in the formation and maintenance of elastic fibers known as microfibrils within the extracellular matrix. Various researchers have suggested that Fibrillin-2 has a role in the mediation of microfibril assembly during early elastogenesis. Defects in Fibrillin-2 are causal to a rare disorder involving autosomal dominant connective tissue known as congenital contractual arachnodactyly (CCA), also known as Beals syndrome or distal arthrogryposis type 9 (DA9), and is similar to Marfan syndrome.

Applications:
Suitable for use in ELISA, Immunohistochemistry, Immunoprecipitation and Immunocytochemistry.
Other applications not tested.

Recommended Dilutions:
Immunohistochemistry (Paraffin): 1:50
Optimal dilutions to be determined by the researcher.

Positive Control:
Liver cancer and papillary serous carcinoma tissue

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.
TypeIsotypeCloneGrade
MabIgG1,k10H6Affinity Purified
SizeStorageShippingSourceHost
100ug-20CBlue IceHumanMouse
Concentration:
~1mg/ml
Immunogen:
Recombinant protein corresponding to human Fibrillin-2.
Purity:
Purified by Protein G affinity chromatography.
Form
Supplied as a liquid in 0.1M Tris-Glycine, pH 7.4, 150mM sodium chloride, 0.05% sodium azide.
Specificity:
Recognizes human Fibrillin-2.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
1.Putnam, E., et al. (1995). Nature Genetics. 11:456-458. 2. Zhang, H., et al. (1994). The journal of Cell Biology. 124:855-863. 3. Quondamatteo, F., et al. (2002). Matrix Biology. 21(8):637-646. 4. Chaudhry, S., et al. (2001). Human Molecular Genetics. 10(8):835-843. 5. Charbonneau, N.L., et al. (2003). The Journal of Biological Chemistry. 278:2740-2749.