WB: Use at a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 50 kDa, and an additional band, of unknown identity, at 23 kDa (predicted molecular weight: 50 kDa).
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
Promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. Could be a vascular ligand for integrin receptors and may play a role in vascular development and remodeling.
Expressed predominantly in heart, ovary, and colon but also in kidney, pancreas, testis, lung and placenta. Not detectable in brain, liver, thymus, prostate, or peripheral blood leukocytes.
Defects in FBLN5 are a cause of autosomal dominant cutis laxa (ADCL) [MIM:123700]. Hereditary cutis laxa refers to a heterogeneous group of connective tissue disorders characterized by cutaneous abnormalities and variable systemic manifestations. The most constant clinical feature is loose skin, sagging over the face and trunk. Hereditary cutis laxa is inherited in both autosomal dominant and autosomal recessive modes. Autosomal dominant cutis laxa is a relatively benign inherited and acquired connective tissue disorder. Defects in FBLN5 are a cause of cutis laxa autosomal recessive type 1 (ARCL1) [MIM:219100]. Hereditary cutis laxa refers to a heterogeneous group of connective tissue disorders characterized by cutaneous abnormalities and variable systemic manifestations. The most constant clinical feature is loose skin, sagging over the face and trunk. Hereditary cutis laxa is inherited in both autosomal dominant and autosomal recessive modes. ARCL1 shows the most severe phenotype and has the poorest prognosis. In addition to the skin, internal organs enriched in elastic fibers, such as the lung and arteries, are affected. Defects in FBLN5 are the cause of age-related macular degeneration type 3 (ARMD3) [MIM:608895]. ARMD is a multifactorial disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane.
Belongs to the fibulin family. Contains 6 EGF-like domains.
Developmental arteries and neural crest EGF like protein antibody
Developmental arteries and neural crest EGF-like protein antibody
FIBL 5 antibody
Urine p50 protein antibody
Western blot - Anti-Fibulin 5 antibody (ab53515)
Anti-Fibulin 5 antibody (ab53515) at 0.1 µg/ml + Human Ovary lysate (35µg protein in RIPA buffer).
Predicted band size: 50 kDa Observed band size: 50 kDa Additional bands at: 23 kDa. We are unsure as to the identity of these extra bands.
Primary incubation was 1 hour. Detected by chemiluminescence.
An additional band of unknown identity was also consistently observed at 23kDa. This band was successfully blocked by incubation with the immunising peptide.