Anti-Fibrillin 1抗体[3H6] (ab124334)


  • 产品名称Anti-Fibrillin 1抗体[3H6]
    参阅全部 Fibrillin 1 一抗
  • 描述
    小鼠单克隆抗体[3H6] to Fibrillin 1
  • 经测试应用适用于: WB, IHC-P, Sandwich ELISAmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant fragment, corresponding to amino acids 2772-2872 of Human Fibrillin 1 with a proprietary tag (NP_000129).

  • 阳性对照
    • Human placenta tissue; Recombinant tagged Fibrillin 1




Our Abpromise guarantee covers the use of ab124334 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
WB 1/500 - 1/1000. Predicted molecular weight: 312 kDa.
IHC-P Use a concentration of 5 µg/ml.
Sandwich ELISA Use at an assay dependent concentration.


  • 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 [3H6] 图像

  • ab124334, at 5 µg/ml staining Fibrillin 1 in formalin-fixed, paraffin-embedded Human Placenta tissue by Immunohistochemistry.
  • Detection limit for recombinant tagged Fibrillin 1 is 0.3 ng/ml as a capture antibody.

Anti-Fibrillin 1 antibody [3H6] (ab124334)参考文献

This product has been referenced in:
  • Pan HT  et al. Differential proteomic analysis of umbilical artery tissue from preeclampsia patients, using iTRAQ isobaric tags and 2D nano LC-MS/MS. J Proteomics 112C:262-273 (2014). Human . Read more (PubMed: 25234496) »

See 1 Publication for this product

Product Wall

Application Western blot
Loading amount 20 µg
Gel Running Conditions Reduced Denaturing (8%)
Sample Human Purified protein (recombinant protein)
Specification recombinant protein
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: RT°C

Miss. Stefanie Heumüller

Verified customer

提交于 Mar 11 2015

Gracias por contactarnos.

Este anticuerpo monoclonal ha sido producido a partir de la proteína humana recombinante comprendida entre los aminoácidos 2771 a 2871. Lamentablemente el epítopo reconocido por el anticuerpo no ...

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