Mouse Anti-Fibrillin 2 (Fibrillin-2, FBN2, Congenital Contractural Arachnodactyly, CCA, DA9) (HRP)
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 sections Optimal dilutions to be determined by the researcher.
Positive Control
Liver cancer and papillary serous carcinoma tissue
Storage and Stability
Store product at 4°C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C. Aliquots are stable at -20°C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
Immunogen
Recombinant protein corresponding to human Fibrillin-2.
Form
Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Horseradish peroxidase (HRP).
Purity
Purified by Protein G affinity chromatography.
Specificity
Recognizes human Fibrillin-2.