货号：AB6065

品牌：Merck Millipore

规格：100Ug

目录价：￥5513.00

市场价格：￥4686.05

会员价格：￥4410.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Fibulin-5 Antibody | AB6065 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Specificity: This antibody recognizes Fibulin-5 near the C-terminus. View All» 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). View All» Epitope: Near C-terminus View All» Immunogen: KLH-conjugated linear peptide corresponding to human Fibulin-5 near the C-terminus. View All» 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). View All» Species Reactivity:

• Mouse

• Human

• Rat

• Chimpanzee

• Rhesus Macaque
  View All» Species Reactivity Note: Demonstrated to react with Mouse, Human, and Rat. Predicted to react with Chimpanzee and Rhesus Macaque based on 100% sequence homology. View All» Control: Mouse heart tissue lysate View All» 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. View All» Purification Method: Affinity Purified View All» Presentation: Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: Q9UBX5 View All» Entrez Gene Number: NP_006320 View All» Gene Symbol:
  • FBLN5

  • DANCE
    View All» Alternate Names:
    • Fibulin-5

    • FIBL-5

    • Developmental arteries and neural crest EGF-like protein

    • Dance

    • Urine p50 protein

    • UP50
      View All» 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. View All» Key Applications: Western Blotting View All» 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]. View All» 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.10Defects 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.9SEQUENCE SIMILARITIES: Belongs to the fibulin family.Contains 6 EGF-like domains. View All» Product Name: Anti-Fibulin-5 View All» Concentration: 1 mg/mL View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Affinity Purified View All» Host: Rabbit View All»
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