Anti-Fibrillin 2 antibody (ab128026)
Key features and details
- Rabbit polyclonal to Fibrillin 2
- Reacts with: Human
- Isotype: IgG
Overview
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Product name
Anti-Fibrillin 2 antibody
See all Fibrillin 2 primary antibodies -
Description
Rabbit polyclonal to Fibrillin 2 -
Host species
Rabbit -
Specificity
No cross-reactivity with Fibrillin 1. -
Species reactivity
Reacts with: Human
Predicted to work with: Cow
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Immunogen
Recombinant fragment containing glycine-rich domain of exon 10 of Human Fibrillin 2.
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Positive control
- Human Prostate, Extra Cellular tissue
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General notes
Reproducibility is key to advancing scientific discovery and accelerating scientists’ next breakthrough.
Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.
We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.
In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.
We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.
Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.
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Properties
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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. -
Storage buffer
Constituents: 99% Whole serum, 1% PBS -
Concentration information loading... -
Purity
Whole antiserum -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
Target
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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-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 disease
Defects 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 similarities
Belongs to the fibrillin family.
Contains 47 EGF-like domains.
Contains 9 TB (TGF-beta binding) domains. -
Cellular localization
Secreted > extracellular space > extracellular matrix. - Information by UniProt
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Database links
- Entrez Gene: 281155 Cow
- Entrez Gene: 2201 Human
- Omim: 121050 Human
- SwissProt: P35556 Human
- Unigene: 519294 Human
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Alternative names
- CCA antibody
- congenital contractural arachnodactyly (Marfanoid-like) antibody
- DA9 antibody
see all
Protocols
Datasheets and documents
References (2)
ab128026 has been referenced in 2 publications.
- Williams JL et al. Defining the molecular signatures of human right heart failure. Life Sci 196:118-126 (2018). PubMed: 29366750
- Gilpin SE et al. Fibrillin-2 and Tenascin-C bridge the age gap in lung epithelial regeneration. Biomaterials 140:212-219 (2017). PubMed: 28662401
