货号：MAB1919

品牌：Merck Millipore

规格：100UL

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 Antibody, clone 11C1.3 | MAB1919 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Bovine zonular fibrils. Reacts well with human elastin microfibrils and can be used to investigate the presence and organization of these fibrils either in Marfan patients or in fibroblasts obtained from these patients. View All» Immunogen: Bovine zonular fibrils View All» Clone: 11C1.3 View All» Isotype: IgG1κ View All» Species Reactivity:

• Bovine

• Human

• Pig
  View All» Application Notes: Immunoblot: antibody reacts with 350,000 Da protein released from bovine aortic smooth muscle cells, which is identical to that of fibrillin. Immunohistochemistry: fresh frozen tissue: 1:200-1:500. Paraffin sections reactive after microwave citrate buffer antigen retrieval. Immunoprecipitation: 5 microliters of antibody diluted in no more than 400 microliters of concentrated supernatants from bovine smooth muscle cells. We recommend either an anti-mouse IgG bead or a rabbit anti-mouse capture antibody followed by protein A. ELISA Optimal dilutions must be determined by the end user. View All» Presentation: Liquid View All» Storage Conditions: Maintain at -20°C in undiluted aliquots for up to 12 months. Avoid repeated freeze/thaw cycles. View All» UniProt Number: P35555 View All» Entrez Gene Number: NM_000138.3 View All» Gene Symbol:
  • FBN1

  • FBN

  • WMS

  • MFS1

  • OCTD

  • MASS

  • SGS
    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:
    • ELISA

    • Immunofluorescence

    • Immunoprecipitation

    • Western Blotting

    • Immunohistochemistry (Paraffin)
      View All» Entrez Gene Summary: This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome. View All» UniProt Summary: FUNCTION: SwissProt: P35555 # 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-1- containing microfibrils provide long-term force bearing structural support.SIZE: 2871 amino acids; 312312 Da SUBUNIT: Interacts with COL16A1.SUBCELLULAR LOCATION: Secreted, extracellular space, extracellular matrix.PTM: Forms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils.DISEASE: SwissProt: P35555 # Defects in FBN1 are a cause of Marfan syndrome (MFS) [MIM:154700]. MFS is an autosomal dominant disorder that affects the skeletal, ocular, and cardiovascular systems. A wide variety of skeletal abnormalities occurs with MFS, including scoliosis, chest wall deformity, tall stature, abnormal joint mobility. Ectopia lentis occurs in up to about 80% of MFS patients and is almost always bilateral. The leading cause of premature death in MFS patients is progressive dilation of the aortic root and ascending aorta, causing aortic incompetence and dissection. The majority of the more than 600 mutations in FBN1 currently known are point mutations, the rest are frameshifts and splice site mutations. Marfan syndrome has been suggested in at least 2 historical figures, Abraham Lincoln and Paganini. & Defects in FBN1 are a cause of isolated ectopia lentis (EL) [MIM:129600]. The symptoms of this autosomal dominant fibrillinopathy overlap with those of Marfan syndrome, with the exclusion of the skeletal and cardiovascular manifestations. & Defects in FBN1 are the cause of autosomal dominant Weill-Marchesani syndrome (WMS) [MIM:608328]. WMS is a rare connective tissue disorder characterized by short stature, brachydactyly, joint stiffness, and eye abnormalities including microspherophakia, ectopia lentis, severe myopia and glaucoma. & Defects in FBN1 are a cause of Shprintzen-Goldberg craniosynostosis syndrome (SGS) [MIM:182212]. SGS is one of a group of disorders characterized by typical features of the Marfan syndrome along with premature closure of the sutures of the skull, causing deformities such as oxycephaly and scaphocephaly. & Defects in FBN1 are a cause of MASS syndrome [MIM:604308]. MASS syndrome is a heritable disorder of connective tissue characterized by involvement of the mitral valve, aorta, skeleton, and skin. MASS syndrome is closely resembling both the Marfan syndrome and the Barlow syndrome. However, no dislocation of the lenses or aneurysmal changes occur in the aorta, and the mitral valve prolapse is by no means invariable.SIMILARITY: SwissProt: P35555 ## Belongs to the fibrillin family. & Contains 47 EGF-like domains. & Contains 7 TGFBP (TGF-beta binding protein) repeats. View All» Brand Family: Chemicon View All» Product Name: Anti-Fibrillin Antibody, clone 11C1.3 | MAB1919 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 µL View All» Format: Purified View All» Host: Mouse View All»
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