货号：AB6066

品牌：Merck Millipore

规格：

目录价：￥4264.00

市场价格：￥3624.40

会员价格：￥3411.20

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Fibulin-6 Antibody | AB6066 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Molecular Weight: 600 kDa calculated. 3 isoforms produced by alternative splicing: Isoform 1 at 613.390 kDa, Isoform 2 at 600.097 kDa, and Isoform 3 at 59.747 kDa. The expected band ~600 kDa is too high for accurate detection by Western Blot; therefore, this antibody is not recommended for this application. View All» Epitope: 0 View All» Immunogen: KLH-conjugated linear peptide corresponding to human Fibulin-6. View All» Background Information: Fibulin-6 (also known as Hemicentin-1) has been linked to age-related macular degeneration. Deregulation of fibulin-6 has been linked to damage of the architecture of the salivary gland in Sjögren's syndrome. Fibulin-6 interacts with ARMS2, which are involved in proper matrix function. View All» Species Reactivity:

• Human

• Mouse

• Rat

• Chimpanzee
  View All» Species Reactivity Note: Demonstrated to react with Human, Mouse, and Rat. Predicted to react with Chimpanzee based on 100% sequence homology. View All» Control: Elastic fibers of human eye tissue or the external laminating membrane of mouse eye tissue. View All» Quality Assurance: Evaluated by immunohistochemistry on human or mouse eye tissues.Immunohistochemistry (paraffin) Analysis: A 1:400 dilution of this antibody was used to detect Fibulin-6 in the elastic fibers of human eye tissue or the external laminating membrane of mouse eye tissue. 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: Q96RW7 View All» Entrez Gene Number: NP_114141 View All» Gene Symbol:
  • FIBL-6

  • HMCN1

  • FIBL6
    View All» Alternate Names:
    • Hemicentin-1

    • Fibulin-6
      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 (Paraffin) View All» Entrez Gene Summary: Summary: This gene encodes a large extracellular member of the immunoglobulin superfamily. A similar protein in C. elegans forms long, fine tracks at specific extracellular sites that are involved in many processes such as stabilization of the germline syncytium, anchorage of mechanosensory neurons to the epidermis, and organization of hemidesmosomes in the epidermis. Mutations in this gene may be associated with age-related macular degeneration. [provided by RefSeq]. View All» UniProt Summary: SUBCELLULAR LOCATION: Secreted › extracellular space › extracellular matrix › basement membrane (by similarity).TISSUE SPECIFICITY: Isoform 1 and isoform 2 are expressed in skin fibroblasts and retinal pigment epithelium (RPE) cells.INVOLVEMENT IN DISEASE: Defects in HMCN1 are a cause of age-related macular degeneration type 1 (ARMD1) [MIM:603075]. ARMD is a multifactorial eye 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.SEQUENCE SIMILARITIES: Contains 7 EGF-like domains. Contains 44 Ig-like C2-type (immunoglobulin-like) domains. Contains 1 nidogen G2 beta-barrel domain. Contains 6 TSP type-1 domains. Contains 1 VWFA domain.SEQUENCE CAUTION: The sequence BAB71154.1 differs from that shown. Reason: Frameshift at position 657. The sequence BAB71216.1 differs from that shown. Reason: Erroneous initiation. View All» Product Name: Anti-Fibulin-6 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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