General description
~ 350 kDa calculated
Fibrillins are glycosolated and disulfide-rich components of the extracellular matrix belonging to the Fibrillin-LTBP protein family. They serve an important function in both nonelastic and elastic tissues such as eye, bone and blood vessels. The functional units of Fibrillin are known as microfibrils, multiprotein assemblies, which act as structural scaffolding for the limiting of the tissue elasticity, structural integrity of different organ systems, the assembly of elastic fibers, and regulation of BMP growth factor signaling. There are three known isoforms; Fibrillin 1, Fibrillin 2, and Fibrillin 3. Fibrillin 3 is the most recently described member of the Fibrillin family, with highest expression in the brain. Alterations in the microfibril assembly often occur in patients with fibrillinopathies such as; Congenital Contractual Arachnodactyly (CCA) which results in disruption of Fibrillin 2, and Marfan’s syndrome resulting from Fibrillin 1 mutation.
Immunogen
Recombinant protein corresponding to human Fibrillin 3.
Application
Anti-Fibrillin (Pan) Antibody, clone 689 detects level of Fibrillin (Pan) & has been published & validated for use in IH, ELISA.
ELISA Analysis: This antibody has been used by an independent laboratory in ELISA (Corson, 2003).
Biochem/physiol Actions
The antibody recognizes isoforms 1, 2, and 3 of fibrillin.
Physical form
Format: Purified
Analysis Note
Control
Mouse skin tissue
Evaluated by Immunohistochemistry in fresh frozen mouse skin tissue.
Immunohistochemistry Analysis: 1:200 dilution of the antibody detected Fibrillin (Pan) in fresh frozen mouse skin tissue.
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
| biological source | mouse |
| Quality Segment | 100 |
| conjugate | unconjugated |
| antibody form | purified immunoglobulin |
| antibody product type | primary antibodies |
| clone | 689, monoclonal |
| species reactivity | mouse |
| species reactivity (predicted by homology) | chicken (based on 100% sequence homology), human (based on 100% sequence homology) |
| technique(s) | ELISA: suitable,immunohistochemistry: suitable |
| isotype | IgG1κ |
| NCBI accession no. | NP_115823 |
| UniProt accession no. | Q75N90 |
| shipped in | wet ice |
| target post-translational modification | unmodified |
| Gene Information | human ... EFEMP1(2202) |
