Fibrillin 1 (FBN1) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Recommend Dilution	Wstern Blot: 1/500-1/1000. Immunohistochemistry on Paraffin Sections: 1/50-1/200.
Reactivity	Human, Mouse, Rat
Host	Rabbit
Clonality	Polyclonal
Immunogen	Synthetic peptide, corresponding to amino acids C-terminus of Human FBN1.
Specificity	This antibody detects endogenous levels of Fibrillin-1 protein. (region surrounding asn2843)
Formulation	Phosphate buffered saline (PBS), pH~7.2 State: Aff - Purified State: Liquid purified Ig fraction (>95% pure by SDS-PAGE) Preservative: 0.05% Sodium Azide
Concentration	1.0 mg/ml
Purification	Affinity Chromatography using epitope-specific immunogen
Conjugation	Unconjugated
Storage Condition	Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Gene Name	fibrillin 1
Database Link	Entrez Gene 2200 Human UniProt ID P35555
Background	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.
Synonyms	FBN1, FBN
Reference Data

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