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Anti-FBN1 Antibody BHA11300303
Species: Homo Sapiens (Human)
Delivery: Free shipping. Usually ships in 7 to 10 business days
Categories: Primary Antibodies|Polyclonal Antibodies
Chromosome Location: 15q21.1
Immunogen: 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.
TARGETWeight: 312 kDa
PROPERTYStorage: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Desc: Product: 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.Specificity: Fibrillin-1 (N2843) polyclonal antibody detects endogenous levels of Fibrillin-1 protein.
Description: Immunohistochemistry (IHC) analyzes of FBN1 (N2843) pAb in paraffin-embedded human breast carcinoma tissue at 1:100.