货号：MAB2644

品牌：Merck Millipore

规格：EA

目录价：￥3696.00

市场价格：￥3141.60

会员价格：￥2956.80

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Fibulin-4 Antibody, clone 226 | MAB2644 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Specificity: The antibody recognizes Fibulin-4. View All» Molecular Weight: ~ 50 kDa. An unknown band at ~ 26 kDa is noted in human placental tissue lysate. View All» Immunogen: Recombinant protein corresponding to human Fibulin-4. View All» Clone: 226 View All» Isotype: IgG1κ View All» Background Information: Fibulin-4 belongs to the Fibulin family, a 6-member, extracellular glycoprotein family characterized by having a unique C-terminal fibulin (FC) domain and a contiguous calcium-binding epidermal growth factor-like domain (cbEGFs). Fibulins have been observed to bind several differing basement membrane proteins, indicating a strong likelihood that they are involved in the stabilization as well as the assembly of ECM structures. Although the function for Fibulin-4 has yet to be fully described, recent studies have shown it to be a necessary component in elastic fiber formation. Fibulin-4 is a widely expressed protein having been observed in skeletal muscle, lung, heart, and placenta.Studies in mice have shown reduced expression of Fibulin-4 to result in cardiac abnormalities, aneurysm formation, and aortic dissection. Additional research has indicated that in patients suffering from osteoarthritis, Fibulin-4 is indicated as a possible target of autoimmunity. View All» Species Reactivity: Human View All» Species Reactivity Note: Demonstrated to react with human. View All» Application Notes: Immunohistochemistry Analysis: 1:500 dilution of this antibody has been shown to detect Fibulin-4 in human embryonic tissue. View All» Control: Human placenta lysate View All» Quality Assurance: Evaluated by Western Blot in human placenta lysate.Western Blot Analysis: 0.5 µg/mL of the antibody detected Fibulin-4 in 10 µg of human placenta lysate. 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: O95967 View All» Entrez Gene Number: NP_058634 View All» Gene Symbol:

• MBP

• FBLN4

• Protein UPH1

• UPH1

• FIBL-4
  View All» Alternate Names:
  • EGF-containing fibulin-like extracellular matrix protein 2

  • fibulin 4

  • Fibulin-4

  • mutant p53 binding protein 1
    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

    • Western Blotting

    • Immunocytochemistry
      View All» Entrez Gene Summary: A large number of extracellular matrix proteins have been found to contain variations of the epidermal growth factor (EGF) domain and have been implicated in functions as diverse as blood coagulation, activation of complement and determination of cell fate during development. The protein encoded by this gene contains four EGF2 domains and six calcium-binding EGF2 domains. This gene is necessary for elastic fiber formation and connective tissue development. Defects in this gene are the cause of an autosomal recessive cutis laxa syndrome. [provided by RefSeq]. View All» UniProt Summary: SUBCELLULAR LOCATION: Secreted. INVOLVEMENT IN DISEASE: Defects in EFEMP2 are a cause of autosomal recessive cutis laxa type I (CL type I) [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. 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.SEQUENCE SIMILARITIES: Belongs to the fibulin family.Contains 6 EGF-like domains. View All» Product Name: Anti-Fibulin-4, clone 226 View All» Concentration: 1.0 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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