Technical Data
F4195-11F
Fibrillin 2 (FBN2)
Description:
Fibrillin-2 is a part of the Fibrillin family of glycoproteins, which are essential factors in the formation and maintenance of elastic fibers known as microfibrils within the extracellular matrix. Various researchers have suggested that Fibrillin-2 has a role in the mediation of microfibril assembly during early elastogenesis. Defects in Fibrillin-2 are causal to a rare disorder involving autosomal dominant connective tissue known as congenital contractual arachnodactyly (CCA), also known as Beals syndrome or distal arthrogryposis type 9 (DA9), and is similar to Marfan syndrome.

Applications:
Suitable for use in ELISA. Other applications not tested.

Recommended Dilution:
ELISA: 1:32,000
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
TypeIsotypeCloneGrade
PabIgGAffinity Purified
SizeStorageShippingSourceHost
100ug-20CBlue IceHumanGoat
Concentration:
~0.5mg/ml
Immunogen:
Synthetic peptide corresponding to C-KELKKLEESNEDD, from the C-terminus of the protein sequence according to NP_001990.2.
Purity:
Purified by immunoaffinity chromatography.
Form
Supplied as a liquid in Tris saline, 0.02% sodium azide, pH7.3, 0.5% BSA.
Specificity:
Recognizes Fibrillin 2. Species sequence homology: human and canine.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
1. Quondamatteo F, Reinhardt DP, Charbonneau NL, Pophal G, Sakai LY, Herken R. Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development. Matrix Biol. 2002 Dec;21(8):637-46. PMID: 12524050.