Western Blot (WB)
(Western blot analysis of Fibrillin-2 expression in LO2 (A), mouse brain (B) whole cell lysates.)
Rabbit anti-Human, Mouse Fibrillin-2 Polyclonal Antibody | anti-FBN2 antibody
Anti-Fibrillin-2 Antibody
Western Blot (WB)
(Western blot analysis of Fibrillin-2 expression in LO2 (A), mouse brain (B) whole cell lysates.)
Western Blot (WB)
(Western blot analysis of Fibrillin-2 expression in LO2 (A), mouse brain (B) whole cell lysates.)
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene is a component of connective tissue microfibrils and may be involved in elastic fiber assembly. Mutations in this gene cause congenital contractural arachnodactyly. [provided by RefSeq, Jul 2008]
Uniprot Description
FBN2: 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. Defects in FBN2 are the cause of distal arthrogryposis type 9 (DA9). A form of distal arthrogryposis, a disease characterized by congenital joint contractures that mainly involve two or more distal parts of the limbs, in the absence of a primary neurological or muscle disease. DA9 is a connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears. Belongs to the fibrillin family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted, signal peptide; Secreted
Chromosomal Location of Human Ortholog: 5q23-q31
Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular region; microfibril
Molecular Function: protein binding; extracellular matrix structural constituent; calcium ion binding
Biological Process: extracellular matrix disassembly; positive regulation of osteoblast differentiation; extracellular matrix organization and biogenesis; anatomical structure morphogenesis; positive regulation of bone mineralization; embryonic limb morphogenesis
Disease: Macular Degeneration, Early-onset; Arthrogryposis, Distal, Type 9
Research Articles on FBN2
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Product Notes
The FBN2 fbn2 (Catalog #AAA8306640) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-Fibrillin-2 Antibody reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's Fibrillin-2 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1:500-1:2000. Researchers should empirically determine the suitability of the FBN2 fbn2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Fibrillin-2, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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