Overview |
bs-12166R-Cy5.5 |
Fibrillin 2 Polyclonal Antibody, Cy5.5 Conjugated |
IF(IHC-P), IF(IHC-F), IF(ICC) |
Human, Mouse, Rat, Dog, Cow, Sheep, Pig, Horse, Chicken, Rabbit |
Specifications |
Cy5.5 |
Rabbit |
KLH conjugated synthetic peptide derived from human Fibrillin 2 |
1001-1200/2912 |
Polyclonal |
IgG |
1ug/ul |
Purified by Protein A. |
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
Target |
Secreted |
CCA; congenital contractural arachnodactyly Marfanoid-like; DA9; FBN2; FBN2_HUMAN; fibrillin 2 congenital contractural arachnodactyly; Fibrillin-2. |
Extracellular glycoproteins fibrillin-1 and -2 are major components of connective tissue microfibrils. Fibrillin-2 containing microfibrils regulate the early process of elastic fiber assembly in tissue. Mutations in the fibrillin-2 gene resulting in impaired assembly of fibrillin-2 may lead to molecular congenital contractural arachnodactyly. Fibrillin-2 constitutes the backbone of microfibrils which insert directly into the lamina densa of basement membranes. Epithelial cells primarily deposit fibrillin into the extracellular matrix in a nonfibrillar form. Mutations in the 8-cysteine motif of Fibrillin-2 alters its binding to microfibril-associated glycoprotein-1 (MAGP-1), which may increase the severity of congenital contractural arachnodactyly. |
Application Dilution |
IF(IHC-P) |
1:50-200 |
IF(IHC-F) |
1:50-200 |
IF(ICC) |
1:50-200 |