Overview

  • Product name

  • Description

    Rabbit polyclonal to Fibrillin 1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Rat, Human
    Predicted to work with: Mouse, Cow, Pig
  • Immunogen

    Recombinant fragment (His-tag) corresponding to Rat Fibrillin 1 aa 168-364. Expressed in E.coli. N-terminal tag.
    Sequence:

    CTYGFTGPQCERDYRTGPCFTVVSNQMCQGQLSGIVCTKTLCCATVGRAW GHPCEMCPAQPHPCRRGFIPNIRTGACQDVDECQAIPGLCQGGNCINTVG SFECKCPAGHKFNEVSQKCEDIDECSTIPGVCDGGECTNTVSSYFCKCPP GFYTSPDGTRCVDVRPGYCYTALTNGRCSNQLPQSITKMQCCCDVGR


    Database link: Q9WUH8

  • Positive control

    • IHC-P: Rat lung tissue. WB: Rat lung and bone marrow lysates; Human lung lysate; Recombinant rat Fibrillin 1 protein.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.40
    Preservative: 0.02% Sodium azide
    Constituents: PBS, 50% Glycerol
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Purification notes

    ab231094 was purified by antigen-specific affinity chromatography followed by Protein A affinity chromatography.
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab231094 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
WB Use a concentration of 0.2 - 2 µg/ml. Predicted molecular weight: 312 kDa.
IHC-P Use a concentration of 5 - 20 µg/ml.

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

Images

  • Paraffin-embedded rat lung tissue stained for Fibrillin 1 using ab231094 at 20 µg/ml in immunohistochemical analysis. DAB staining.

  • Anti-Fibrillin 1 antibody (ab231094) at 1 µg/ml + Rat lung lysate

    Predicted band size: 312 kDa

  • Anti-Fibrillin 1 antibody (ab231094) at 2 µg/ml + Rat bone marrow lysate

    Predicted band size: 312 kDa

  • Anti-Fibrillin 1 antibody (ab231094) at 2 µg/ml + Human lung lysate

    Predicted band size: 312 kDa

  • Anti-Fibrillin 1 antibody (ab231094) at 2 µg/ml + Recombinant rat Fibrillin 1 protein

    Predicted band size: 312 kDa

References

ab231094 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Application
Western blot
Sample
Mouse Cell lysate - whole cell (Cell Lines)
Gel Running Conditions
Reduced Denaturing
Loading amount
30 µg
Specification
Cell Lines
Blocking step
Milk as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 25°C

Qiong ZHOU

Verified customer

Submitted May 28 2019

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