merck millipore,默克密理愽
  • merck millipore,默克密理博,MAB2502,Anti-Fibrillin-1 Antibody, NT, clone 26

  • merck millipore,默克密理博,MAB2502,Anti-Fibrillin-1 Antibody, NT, clone 26

    产品名称：Anti-Fibrillin-1 Antibody, NT, clone 26
    产品型号：MAB2502
    Anti-Fibrillin-1 Antibody, N-terminus, clone 26 detects level of Fibrillin-1 & has been published & validated for use in ELISA, IP, WB & IC.

    merck millipore,默克密理博,MAB2502,Anti-Fibrillin-1 Antibody, NT, clone 26

  • 产品介绍
  • merck millipore,默克密理博,MAB2502,Anti-Fibrillin-1 Antibody, NT, clone 26

    重要规格表

    品种反应性 主要应用 宿主 格式 抗体类型
    HICC, ELISA, WBMPurifiedMonoclonal Antibody
    描述
    产品目录编号 MAB2502
    品牌系列 Chemicon®
    商名
    • Chemicon
    描述 Anti-Fibrillin-1 Antibody, NT, clone 26
    产品信息
    格式 Purified
    演示 Liquid at 1 mg/mL in 20 mM phosphate buffer, 250 mM NaCl, pH 7.6, containing 0.1% sodium azide.Note: Sodium azide is toxic. MSDS available upon request.
    应用
    应用 Anti-Fibrillin-1 Antibody, N-terminus, clone 26 detects level of Fibrillin-1 & has been published & validated for use in ELISA, IP, WB & IC.
    主要应用
    • Immunocytochemistry
    • ELISA
    • Western Blotting
    应用说明 Immunoblotting

    Immunofluorescence

    Immunoprecipitation

    ELISA

    Optimal working dilutions must be determined by end user.
    生物信息
    免疫原品种 Human Fibrillin-1
    表位 N-terminus
    克隆 26
    宿主 Mouse
    特异性 Monoclonal antibody MAB2502 recognizes human Fibrillin-1. Epitope mapping studies identify the binding site of this antibody to amino-terminal end of the molecule, between amino acid residues 45 and 450. The antibody is reactive with human, chicken, and bovine Fibrillin-1.
    同种型 IgG1
    品种反应性 Human
    抗体类型 Monoclonal Antibody
    Entrez基因编号
    • NM_000138.3
    Entrez基因汇总 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.
    基因符号
    • SGS
    • FBN1
    • FBN
    • MASS
    • WMS
    • MFS1
    • OCTD
    UniProt编号
    • P35555
    UniProt汇总 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.
    产品使用声明
    使用声明
    • 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.
    储存和货运信息
    存储条件 Maintain refrigerated at 2-8°C in undiluted aliquots for up to 12 months.
    包装信息
    数量 100 µg

    merck millipore,默克密理博,MAB2502,Anti-Fibrillin-1 Antibody, NT, clone 26

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