Technical Data
F4194
Fibrillin 1
Description:
Fibrillin-1 is an extracellular acidic protein with a high cysteine content and an extended thread-like shape with mosaic composition of different types of extracellular modules. Most of the fibrillin molecule is contributed by 47 epidermal growth factor-like (EGF-like) repeats; 43 of them have a consensus sequence for calcium binding (cb). Other modules including 8-cysteine motifs (seven), hybrid motifs (two), and unique domains (three) are interspersed throughout the molecule. Fibrillin-1 contains a proline-rich region close to the N-terminus that is replaced by a glycine-rich region in fibrillin-2. The fibrillins are found throughout the connective tissue as integral components of extended fibrils. There is an aberrant expression of fibrillin-1 in Marfan syndrome.

Applications: Suitable for use in Immunoblot, Immunoprecipitation, ELISA.

Storage and Handling: Maintain at-20C in undiluted aliquots for up to 12 months. Avoid repeated freeze/thaw cycles.
TypeIsotypeCloneGrade
MabIgG1,k2Q629Ascites
SizeStorageShippingSourceHost
100ul-20CBlue IceBovineMouse
Concentration:
Not determined
Immunogen:
Bovine zonular fibrils
Purity:
Ascites
Form
Ascites
Specificity:
Bovine zonular fibrils. Reacts well with human elastin microfibrils and can be used to investigate the presence and organization of these fibrils either in Marfan patients or in fibroblasts obtained from these patients.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
Wright et al., Immunization with undernatured bovine zonular fibrils results in antibodies to fibrillin. Matrix Biology 14: 4149 (1994).