Product nameAnti-Fibrillin 2 antibody
See all Fibrillin 2 primary antibodies
DescriptionRabbit polyclonal to Fibrillin 2
SpecificityNo cross-reactivity with Fibrillin 1.
Tested applicationsSuitable for: IHC-P, IP, ICC, WB, ELISAmore details
Species reactivityReacts with: Human
Predicted to work with: Cow
Recombinant fragment containing glycine-rich domain of exon 10 of Human Fibrillin 2.
- Human Prostate, Extra Cellular tissue
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term.
Storage bufferConstituents: 99% Whole serum, 1% PBS
Our Abpromise guarantee covers the use of ab128026 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IP||1/100 - 1/200.|
|ICC||1/50 - 1/100.|
|WB||1/200 - 1/500. Predicted molecular weight: 315 kDa.|
|ELISA||1/1000 - 1/2000.|
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
This product has been referenced in:
- Williams JL et al. Defining the molecular signatures of human right heart failure. Life Sci 196:118-126 (2018). Read more (PubMed: 29366750) »
- Gilpin SE et al. Fibrillin-2 and Tenascin-C bridge the age gap in lung epithelial regeneration. Biomaterials 140:212-219 (2017). Read more (PubMed: 28662401) »