Product Information
83836-4-PBS targets Fibrillin 1 in WB, IHC, ELISA applications and shows reactivity with human samples.
Tested Reactivity | human |
Host / Isotype | Rabbit / IgG |
Class | Recombinant |
Type | Antibody |
Immunogen | Fibrillin 1 fusion protein Ag30283 Predict reactive species |
Full Name | fibrillin 1 |
Observed Molecular Weight | 350 kDa |
GenBank Accession Number | BC146854 |
Gene Symbol | Fibrillin 1 |
Gene ID (NCBI) | 2200 |
Conjugate | Unconjugated |
Form | Liquid |
Purification Method | Protein A purfication |
UNIPROT ID | P35555 |
Storage Buffer | PBS Only |
Storage Conditions | Store at -80°C. |
Background Information
Fibrillin-1 belongs to the fibrillin family. Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1-containing microfibrils provide long-term force bearing structural support. Defects in Fibrillin-1 are a cause of Marfan syndrome (MFS), isolated ectopia lentis (EL), Weill-Marchesani syndrome autosomal dominant (ADWMS), Shprintzen-Goldberg craniosynostosis syndrome (SGS) or MASS syndrome.