Recombinant Human FBN1

• Cat.No.: FBN1-28892TH
• Product Overview: Recombinant fragment corresponding to amino acids 2772-2871 of Human Fibrillin 1 with an N terminal proprietary tag; Predicted MWt 36.63 kDa.

Specification

• Description: This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome.
• Protein length: 100 amino acids
• Molecular Weight: 36.630kDa inclusive of tags
• Source: Wheat germ
• Form: Liquid
• Purity: Proprietary Purification
• Storage buffer: pH: 8.00Constituents:0.3% Glutathione, 0.79% Tris HCl
• Storage: Shipped on dry ice. Upon delivery aliquot and store at -80oC. Avoid freeze / thaw cycles.
• Sequences of amino acids: SNKVRILELLPALTTLTNHNRYLIE SGNEDGFFKINQKEGISYLHFTKKK PVAGTYSLQISSTPLYKKKELNQLE DKYDKDYLSGELGDNLKMKIQVLLH
• Sequence Similarities: Belongs to the fibrillin family.Contains 47 EGF-like domains.Contains 9 TB (TGF-beta binding) domains.

Gene Information

• Gene Name: FBN1 fibrillin 1 [ Homo sapiens ]
• Official Symbol: FBN1
• Synonyms: FBN1; fibrillin 1; FBN, fibrillin 1 (Marfan syndrome) , MFS1, WMS; fibrillin-1; Marfan syndrome; MASS; OCTD; SGS
• Gene ID: 2200
• mRNA Refseq: NM_000138
• Protein Refseq: NP_000129
• MIM: 134797
• Uniprot ID: P35555
• Chromosome Location: 15q21.1
• Pathway: Integrin cell surface interactions, organism-specific biosystem; Signal Transduction, organism-specific biosystem
• Function: calcium ion binding; extracellular matrix structural constituent; protein binding