Overview

  • Product name
  • Description
    Rabbit polyclonal to Fibrillin 1
  • Specificity
    Fibrillin 1 antibody detects endogenous levels of total Fibrillin 1 protein.
  • Tested applications
    Suitable for: ICC/IF, IHC-P, ELISAmore details
  • Species reactivity
    Reacts with: Mouse, Human
    Predicted to work with: Rat
  • Immunogen

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

  • Positive control
    • Human breast carcinoma tissue

Properties

  • Form
    Liquid
  • Storage instructions
    Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
  • Storage buffer
    Preservative: 0.02% Sodium Azide
    Constituents: 50% Glycerol, PBS (without Mg2+ and Ca2+), 150mM Sodium chloride, pH 7.4
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Purification notes
    The antibody was affinity purified from rabbit antiserum by affinity chromatography using epitope-specific immunogen.
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • Research areas

Applications

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.

Application Abreviews Notes
ICC/IF Use at an assay dependent concentration. PubMed: 23401661
IHC-P Use at an assay dependent concentration.
ELISA 1/40000.

Target

  • Function
    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.
  • Involvement in disease
    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.
  • Sequence similarities
    Belongs to the fibrillin family.
    Contains 47 EGF-like domains.
    Contains 9 TB (TGF-beta binding) domains.
  • Post-translational
    modifications
    Forms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils.
  • Cellular localization
    Secreted > extracellular space > extracellular matrix.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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 images

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

References for Anti-Fibrillin 1 antibody (ab53076)

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
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Submitted Feb 19 2016

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