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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| M, H, R, Chp, Rhesus Macaque | WB | Rb | Affinity Purified | Polyclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | AB6065 |
| Description | Anti-Fibulin-5 Antibody |
| Alternate Names |
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| Background Information | Fibulin-5 (also known as EVEC/DANCE) is an extracellular matrix protein. Fibulin-5 is most commonly found in blood vessels and cardiac valves during embryogenesis and in adult tissue that contains elastic fibers like the skin, lungs, uterus and aorta. Fibulin-5 travels to the surface of the elastic fibers and its activity is dependent on calcium. Fibulin-5 seems to work as a scaffold protein that facilitates the binding of the elastic fibers to cells and the formation of the elastic fibers themselves. Mutations of fibulin-5 have been associated with age-related macular degeneration (AMD) and cutis laxa (CL). |
| Product Information | |
|---|---|
| Format | Affinity Purified |
| Control |
|
| Presentation | Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Fibulin-5 Antibody detects level of Fibulin-5 & has been published & validated for use in WB. |
| Key Applications |
|
| Biological Information | |
|---|---|
| Immunogen | KLH-conjugated linear peptide corresponding to human Fibulin-5 near the C-terminus. |
| Epitope | Near C-terminus |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Rabbit |
| Specificity | This antibody recognizes Fibulin-5 near the C-terminus. |
| Species Reactivity |
|
| Species Reactivity Note | Demonstrated to react with Mouse, Human, and Rat. Predicted to react with Chimpanzee and Rhesus Macaque based on 100% sequence homology. |
| Antibody Type | Polyclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | The protein encoded by this gene is a secreted, extracellular matrix protein containing an Arg-Gly-Asp (RGD) motif and calcium-binding EGF-like domains. It promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. It is prominently expressed in developing arteries but less so in adult vessels. However, its expression is reinduced in balloon-injured vessels and atherosclerotic lesions, notably in intimal vascular smooth muscle cells and endothelial cells. Therefore, the protein encoded by this gene may play a role in vascular development and remodeling. Defects in this gene are a cause of autosomal dominant cutis laxa, autosomal recessive cutis laxa type I (CL type I), and age-related macular degeneration type 3 (ARMD3). [provided by RefSeq]. |
| Gene Symbol |
|
| Purification Method | Affinity Purfied |
| UniProt Number |
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| UniProt Summary | FUNCTION: 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. SUBUNIT STRUCTURE: Homodimer. SUBCELLULAR LOCATION: Secreted. TISSUE SPECIFICITY: 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. INVOLVEMENT IN DISEASE: 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. Ref.7 Ref.10 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. Ref.9 SEQUENCE SIMILARITIES: Belongs to the fibulin family. Contains 6 EGF-like domains. |
| Molecular Weight | ~54 kDa observed. The calculated molecular weight is 50 kDa; however bands have been observed at ~56 kDa (Freeman, L.J., et al. (2005) Biochem. J. 388:1-5). |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Western Blot in mouse heart tissue lysate. Western Blot Analysis: 0.1 µg/mL of this antibody detected Fibulin-5 in 10 µg of mouse heart tissue lysate. |
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |