概述

  • 产品名称Anti-Fibrillin 1抗体
    参阅全部 Fibrillin 1 一抗
  • 描述
    兔多克隆抗体to Fibrillin 1
  • 特异性Fibrillin 1 antibody detects endogenous levels of total Fibrillin 1 protein.
  • 经测试应用适用于: ICC/IF, IHC-P, ELISAmore details
  • 种属反应性
    与反应: Mouse, Human
    预测可用于: Rat
  • 免疫原

    Synthetic peptide: STPLYKKKEL NQLEDKYDKD YLSGELGDNL KMKIQVLLH, corresponding to amino acids 2832-2871 of Human Fibrillin 1

  • 阳性对照
    • Human breast carcinoma tissue

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
  • 存储溶液Preservative: 0.02% Sodium Azide
    Constituents: 50% Glycerol, PBS (without Mg2+ and Ca2+), 150mM Sodium chloride, pH 7.4
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 纯化说明The antibody was affinity purified from rabbit antiserum by affinity chromatography using epitope-specific immunogen.
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab53076 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
ICC/IF Use at an assay dependent concentration. PubMed: 23401661
IHC-P Use at an assay dependent concentration.
ELISA 1/40000.

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 图像

  • Immunohistochemical analysis of paraffin-embedded human breast carcinoma tissue
    using ab53076 at 1/50 dilution, with and without immunizing peptide.

Anti-Fibrillin 1 antibody (ab53076)参考文献

This product has been referenced in:
  • Pérez-Rico C  et al. Elastin development-associated extracellular matrix constituents of subepithelial connective tissue in human pterygium. Invest Ophthalmol Vis Sci 55:6309-18 (2014). Human . Read more (PubMed: 25183766) »
  • Jelodari-Mamaghani S  et al. Contribution of the latent transforming growth factor-beta binding protein 2 gene to etiology of primary open angle glaucoma and pseudoexfoliation syndrome. Mol Vis 19:333-47 (2013). ICC/IF ; Human . Read more (PubMed: 23401661) »

See all 4 Publications for this product

Product Wall

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample Mouse Tissue sections (aorta)
Antigen retrieval step Heat mediated - Buffer/Enzyme Used: Citrate buffer pH=6
Permeabilization No
Specification aorta
Blocking step Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: RT°C
Fixative 10% formalin
Username

Abcam user community

Verified customer

提交于 Feb 19 2016

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"