ITM0273
ITM0273
ITM0273
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- Catalog: ITM0273
- Gene/Protein: FBLN5
- Product Description: Immunotag™ Fibulin-5 Monoclonal Antibody
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Description
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Specifications
Antibody Specification Datasheet 
IMPORTANT NOTE This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. Target Protein Fibulin-5 Clonality Monoclonal Storage/Stability -20°C/1 year Application WB,ELISA Recommended Dilution Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications. Concentration 1 mg/ml Reactive Species Human Host Species Mouse Immunogen Purified recombinant fragment of Fibulin-5 expressed in E. Coli. Specificity Fibulin-5 Monoclonal Antibody detects endogenous levels of Fibulin-5 protein. Purification Affinity purification Form Ascitic fluid containing 0.03% sodium azide. Gene Name FBLN5 Accession No. Q9UBX5 Q9WVH9 Alternate Names FBLN5; DANCE; Fibulin-5; FIBL-5; Developmental arteries and neural crest EGF-like protein; Dance; Urine p50 protein; UP50 Description fibulin 5(FBLN5) Homo sapiens 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, Jul 2008], Protein Expression Lung,Melanoma,Placenta,Urine, Subcellular Localization extracellular region,proteinaceous extracellular matrix,extracellular space,extracellular matrix,extracellular exosome,elastic fiber, Protein Function disease:Defects in FBLN5 are a cause of autosomal dominant cutis laxa [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.,disease:Defects in FBLN5 are a cause of autosomal recessive cutis laxa type I (CL type I) [MIM:219100]. CL type I 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.,disease: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.,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.,similarity:Belongs to the fibulin family.,similarity:Contains 6 EGF-like domains.,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., Usage For Research Use Only! Not for diagnostic or therapeutic procedures. - Reviews
| Antibody Specification | |
| Datasheet | |
| IMPORTANT NOTE | This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. |
| Target Protein | Fibulin-5 |
| Clonality | Monoclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,ELISA |
| Recommended Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human |
| Host Species | Mouse |
| Immunogen | Purified recombinant fragment of Fibulin-5 expressed in E. Coli. |
| Specificity | Fibulin-5 Monoclonal Antibody detects endogenous levels of Fibulin-5 protein. |
| Purification | Affinity purification |
| Form | Ascitic fluid containing 0.03% sodium azide. |
| Gene Name | FBLN5 |
| Accession No. | Q9UBX5 Q9WVH9 |
| Alternate Names | FBLN5; DANCE; Fibulin-5; FIBL-5; Developmental arteries and neural crest EGF-like protein; Dance; Urine p50 protein; UP50 |
| Description | fibulin 5(FBLN5) Homo sapiens 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, Jul 2008], |
| Protein Expression | Lung,Melanoma,Placenta,Urine, |
| Subcellular Localization | extracellular region,proteinaceous extracellular matrix,extracellular space,extracellular matrix,extracellular exosome,elastic fiber, |
| Protein Function | disease:Defects in FBLN5 are a cause of autosomal dominant cutis laxa [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.,disease:Defects in FBLN5 are a cause of autosomal recessive cutis laxa type I (CL type I) [MIM:219100]. CL type I 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.,disease: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.,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.,similarity:Belongs to the fibulin family.,similarity:Contains 6 EGF-like domains.,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., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |