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AB6065 Anti-Fibulin-5 Antibody

Anti-Fibulin-5 Antibody detects level of Fibulin-5 & has been published & validated for use in WB.

Anti-Fibulin-5 Antibody MSDS (material safety data sheet) or SDS, CoA and CoQ, dossiers, brochures and other available documents.
Research Category: Cell Structure Research Sub-Category: ECM Proteins Gene Symbol: FBLN5, DANCE UniProt Number: Q9UBX5
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AB6065
100 µg  
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      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      M, H, R, Chp, Rhesus MacaqueWBRbAffinity PurifiedPolyclonal Antibody
      Description
      Catalogue NumberAB6065
      DescriptionAnti-Fibulin-5 Antibody
      Alternate Names
      • Fibulin-5
      • FIBL-5
      • Developmental arteries and neural crest EGF-like protein
      • Dance
      • Urine p50 protein
      • UP50
      Background InformationFibulin-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).
      References
      Product Information
      FormatAffinity Purified
      Control
      • Mouse heart tissue lysate
      PresentationPurified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationAnti-Fibulin-5 Antibody detects level of Fibulin-5 & has been published & validated for use in WB.
      Key Applications
      • Western Blotting
      Biological Information
      ImmunogenKLH-conjugated linear peptide corresponding to human Fibulin-5 near the C-terminus.
      EpitopeNear C-terminus
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostRabbit
      SpecificityThis antibody recognizes Fibulin-5 near the C-terminus.
      Species Reactivity
      • Mouse
      • Human
      • Rat
      • Chimpanzee
      • Rhesus Macaque
      Species Reactivity NoteDemonstrated to react with Mouse, Human, and Rat.
      Predicted to react with Chimpanzee and Rhesus Macaque based on 100% sequence homology.
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThe 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
      • FBLN5
      • DANCE
      Purification MethodAffinity Purfied
      UniProt Number
      UniProt SummaryFUNCTION: 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).
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated 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
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Catalogue Number GTIN
      AB6065 04053252293764