Anti-Fibrillin 1抗体[11C1.3] (Biotin) (ab24826)

概述

  • 产品名称Anti-Fibrillin 1抗体[11C1.3] (Biotin)
    参阅全部 Fibrillin 1 一抗
  • 描述
    小鼠单克隆抗体[11C1.3] to Fibrillin 1 (Biotin)
  • 偶联物Biotin
  • 特异性This antibody recognises fibrillin (Mr 350 kDa).
  • 经测试应用适用于: IHC-Fr, IHC-P, ICC/IF, Electron Microscopy, WBmore details
  • 种属反应性
    与反应: Cow, Human
  • 免疫原

    Microfibrils from zonular apparatus of bovine eye.

性能

应用

Our Abpromise guarantee covers the use of ab24826 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
IHC-Fr
IHC-P
ICC/IF
Electron Microscopy
WB
  • 应用说明Electron Microscopy: Use at an assay dependent dilution.
    IF: Use at an assay dependent dilution.
    IHC-P: 1/200. Perform heat mediated antigen retrieval via the microwave/citrate buffer method before commencing with IHC staining protocol.
    IHC-Fr: 1/200 - 1/500.
    WB: Use at a concentration of 1 µg/ml. Predicted molecular weight: 312 kDa.


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 功能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. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.
    • 疾病相关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. Note=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 Weill-Marchesani syndrome autosomal dominant (ADWMS) [MIM:608328]. 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 a very rare syndrome characterized by a marfanoid habitus, craniosynostosis, characteristic dysmorphic facial features, skeletal and cardiovascular abnormalities, mental retardation, developmental delay and learning disabilities.
      Defects in FBN1 are a cause of overlap connective tissue disease (OCTD) [MIM:604308]. 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.
      Defects in FBN1 are a cause of stiff skin syndrome (SSKS) [MIM:184900]. It is a syndrome characterized by hard, thick skin, usually over the entire body, which limits joint mobility and causes flexion contractures. Other occasional findings include lipodystrophy and muscle weakness.
    • 序列相似性Belongs to the fibrillin family.
      Contains 47 EGF-like domains.
      Contains 9 TB (TGF-beta binding) domains.
    • 翻译后修饰Forms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils.
    • 细胞定位Secreted > extracellular space > extracellular matrix.
    • Information by UniProt
    • 数据库链接
    • 别名
      • 350 kDa glycoprotein component extracellular microfibril antibody
      • ACMICD antibody
      • FBN 1 antibody
      • FBN antibody
      • FBN1 antibody
      • FBN1_HUMAN antibody
      • Fibrillin 15 antibody
      • Fibrillin-1 antibody
      • Fibrillin1 antibody
      • Fibrillin15 antibody
      • GPHYSD2 antibody
      • Marfan syndrome antibody
      • MASS antibody
      • MFS 1 antibody
      • MFS1 antibody
      • OCTD antibody
      • SGS antibody
      • SSKS antibody
      • Weill Marchesani syndrome antibody
      • WMS antibody
      • WMS2 antibody
      see all

    Anti-Fibrillin 1 antibody [11C1.3] (Biotin) (ab24826)参考文献

    This product has been referenced in:
    • Maier A  et al. Fibrillin and elastin networks in extrafusal tissue and muscle spindles of bovine extraocular muscles. Invest Ophthalmol Vis Sci 35:3103-10 (1994). Read more (PubMed: 8206729) »
    • Wright DW  et al. Immunization with undenatured bovine zonular fibrils results in monoclonal antibodies to fibrillin. Matrix Biol 14:41-9 (1994). Read more (PubMed: 8061919) »

    See all 2 Publications for this product

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