BS1127

antibody from Bioworld Technology, Inc

Targeting: FBN1 FBN, MASS, MFS1, OCTD, SGS, WMS

Immunohistochemistry

Antibody data

Submit

Validation data

Product number: BS1127 - Provider product page
Provider: Bioworld Technology, Inc
Proper citation: Bioworld Technology Cat#BS1127, RRID:AB_1662062
Product name: FBN1 (N2843) polyclonal antibody
Antibody type: Polyclonal
Antigen: Synthetic peptide, corresponding to amino acids C-terminus of Human FBN1.
Description: 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.
Reactivity: Human, Mouse, Rat
Host: Rabbit
Isotype: IgG
Vial size: 100ul
Concentration: 1 mg/ml
Storage: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

FBN1 protein structure - BS1127 shown in red.