货号：MAB2641

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-Fibrillin (Pan) Antibody, clone 689 | MAB2641 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Specificity: The antibody recognizes isoforms 1, 2, and 3 of fibrillin. View All» Molecular Weight: ~ 350 kDa calculated View All» Immunogen: Recombinant protein corresponding to human Fibrillin 3. View All» Clone: 689 View All» Isotype: IgG1κ View All» Background Information: Fibrillins are glycosolated and disulfide-rich components of the extracellular matrix belonging to the Fibrillin-LTBP protein family. They serve an important function in both nonelastic and elastic tissues such as eye, bone and blood vessels. The functional units of Fibrillin are known as microfibrils, multiprotein assemblies, which act as structural scaffolding for the limiting of the tissue elasticity, structural integrity of different organ systems, the assembly of elastic fibers, and regulation of BMP growth factor signaling. There are three known isoforms; Fibrillin 1, Fibrillin 2, and Fibrillin 3. Fibrillin 3 is the most recently described member of the Fibrillin family, with highest expression in the brain. Alterations in the microfibril assembly often occur in patients with fibrillinopathies such as; Congenital Contractual Arachnodactyly (CCA) which results in disruption of Fibrillin 2, and Marfan’s syndrome resulting from Fibrillin 1 mutation. View All» Species Reactivity:

• Mouse

• Human

• Chicken
  View All» Species Reactivity Note: Demonstrated to react with mouse. Predicted to react with human and chicken based on 100% sequence homology. View All» Application Notes: ELISA Analysis: This antibody has been used by an independent laboratory in ELISA (Corson, 2003). View All» Control: Mouse skin tissue View All» Quality Assurance: Evaluated by Immunohistochemistry in fresh frozen mouse skin tissue.Immunohistochemistry Analysis: 1:200 dilution of the antibody detected Fibrillin (Pan) in fresh frozen mouse skin tissue. View All» Purification Method: Protein G purfied View All» Presentation: Purified mouse monoclonal IgG1κ 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: Q75N90 View All» Entrez Gene Number: NP_115823 View All» Gene Symbol:
  • EFEMP1

  • FBNL

  • MLVT

  • FBLN3

  • RAD

  • fibrillin-like

  • Fibulin-3

  • S1-5

  • FIBL-3

  • HRD

  • MTLV
    View All» Alternate Names:
    • EGF-containing fibulin-like extracellular matrix protein 1

    • Fibrillin-like protein

    • Extracellular protein S1-5
      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:
      • Immunohistochemistry

      • ELISA
        View All» Entrez Gene Summary: This gene encodes a protein that belongs to the fibrillin gene family. Fibrillins are extracellular matrix molecules that assemble into microfibrils in many connective tissues. This gene is most highly expressed in fetal tissues and its protein product is localized to extracellular microfibrils of developing skeletal elements, skin, lung, kidney, and skeletal muscle. This gene is potentially involved in Weill-Marchesani syndrome. While several transcript variants may exist for this gene, their full-length natures have not been described to date. [provided by RefSeq]. View All» UniProt Summary: FUNCTION: Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-containing microfibrils provide long-term force bearing structural support. SUBCELLULAR LOCATION: Secreted › extracellular space › extracellular matrixTISSUE SPECIFICITY: Predominantly expressed in connective tissues such as skeletal muscle, tendon, skin, perichondrium and periosteum. Highly expressed in fetal lung, brain, kidney. Expressed at low level in prostate, testis, mammary gland, uterus, ovary, placenta, bladder, adrenal gland, thyroid, fetal thymus, fetal liver, liver, fetal heart and heart. PTM: Probably forms intermolecular disulfide bonds either with other FBN3 molecules or with other components of the microfibrils (by similarity).SEQUENCE SIMILARITIES: Belongs to the fibrillin family.Contains 44 EGF-like domains.Contains 9 TB (TGF-beta binding) domains.SEQUENCE CAUTION: The sequence BAB47408.2 differs from that shown. Reason: Erroneous initiation. View All» Product Name: Anti-Fibrillin (Pan), clone 689 View All» Concentration: 0.5 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Mouse View All»
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