Key features and details
- Rabbit polyclonal to Fibrillin 2
- Suitable for: IHC-P, WB
- Reacts with: Human
- Isotype: IgG
Product nameAnti-Fibrillin 2 antibody
See all Fibrillin 2 primary antibodies
DescriptionRabbit polyclonal to Fibrillin 2
Tested applicationsSuitable for: IHC-P, WBmore details
Species reactivityReacts with: Human
- IHC: Human placenta tissue. WB: U-2 OS cell lysate.
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Storage instructionsShipped 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 bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab224256 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/50 - 1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
|WB||Use a concentration of 0.04 - 0.4 µg/ml. Predicted molecular weight: 315 kDa.|
FunctionFibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2-containing microfibrils regulate the early process of elastic fiber assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.
Involvement in diseaseDefects in FBN2 are the cause of congenital contractural arachnodactyly (CCA) [MIM:121050]; also known as Beals syndrome or distal arthrogryposis type 9 (DA9). CCA is a rare, autosomal dominant connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears. Phenotypically similar to Marfan syndrome, CCA does not affect the aorta and the eyes.
Sequence similaritiesBelongs to the fibrillin family.
Contains 47 EGF-like domains.
Contains 9 TB (TGF-beta binding) domains.
Cellular localizationSecreted > extracellular space > extracellular matrix.
- Information by UniProt
- CCA antibody
- congenital contractural arachnodactyly (Marfanoid-like) antibody
- DA9 antibody
Paraffin-embedded human placenta tissue stained for Fibrillin 2 using ab224256 at 1/50 dilution in immunohistochemical analysis.
Anti-Fibrillin 2 antibody (ab224256) at 1/100 dilution + U-2 OS (human bone osteosarcoma epithelial cell line) cell lysate
Developed using the ECL technique.
Predicted band size: 315 kDa
ab224256 has not yet been referenced specifically in any publications.