The fibrillin family of proteins, including fibrillin-1 (FBN1) and fibrillin-2 (FBN2), are integral components of a distinct subset of extracellular microfibrils.
Microfibrils are found in elastic tissues where they facilitate elastic fiber formation and in nonelastic tissue where they support the association of the epithelial cells with the interstitial matrix.
Characteristic of the fibrillin proteins are the epidermal growth factor (EGF)-like motifs, which contain a consensus sequence for calcium binding.
This calcium association may be critical for protein-protein interactions and stabilization of the microfibrils.
Mutations of the FBN1 gene have been shown to result in Marfan syndrome, a disease characterized by abnormal synthesis, secretion and matrix deposition of fibrillin.
FBN2 is also linked to a rare, yet similiar, skeletal disorder, congenital contractural arachnodactyly.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:1000Immunohistochemistry: 1:50-1:200Optimal 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.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.05% sodium azide, pH 7.2.
Specificity：Recognizes endogenous levels of FBN1. Species Crossreactivity: Human, mouse, rat
Isotype：IgG
Calc Applications Abbrev：IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to amino acids C-terminus of Human FBN1.
