Rabbit EXT2 Polyclonal Antibody | anti-EXT2 antibody
EXT2 Antibody (Center)
IHC-P~~1:10~50
IF~~1:10~50
FC~~1:10~50
Western Blot (WB)
(Western blot analysis of lysate from T47D cell line,using EXT2 Antibody (Center). MBS9215203 was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:5000 dilution was used as the secondary antibody.Lysate at 35ug per lane.)
Immunohistochemistry (IHC)
(Formalin-fixed and paraffin-embedded human brain tissue reacted with EXT2 Antibody (Center), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)
Immunofluorescence (IF)
(Confocal immunofluorescent analysis of EXT2 Antibody (Center) with hela cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).)
Flow Cytometry (FC/FACS)
(EXT2 Antibody (Center) flow cytometry analysis of Hela cells (bottom histogram) compared to a negative control cell (top histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)
Li, Y., et al. Genet Test Mol Biomarkers 13(6):825-830(2009)
Yerges, L.M., et al. J. Bone Miner. Res. 24(12):2039-2049(2009)
Heinritz, W., et al. Ann. Hum. Genet. 73 (PT 3), 283-291 (2009)
Cornelis, M.C., et al. Ann. Intern. Med. 150(8):541-550(2009)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes one of two glycosyltransferases involved in the chain elongation step of heparan sulfate biosynthesis. Mutations in this gene cause the type II form of multiple exostoses. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008]
Uniprot Description
EXT2: Glycosyltransferase required for the biosynthesis of heparan-sulfate. The EXT1/EXT2 complex possesses substantially higher glycosyltransferase activity than EXT1 or EXT2 alone. Appears to be a tumor suppressor. Defects in EXT2 are a cause of hereditary multiple exostoses type 2 (EXT2). EXT is a genetically heterogeneous bone disorder caused by genes segregating on human chromosomes 8, 11, and 19 and designated EXT1, EXT2 and EXT3 respectively. EXT is a dominantly inherited skeletal disorder primarily affecting endochondral bone during growth. The disease is characterized by formation of numerous cartilage-capped, benign bone tumors (osteocartilaginous exostoses or osteochondromas) that are often accompanied by skeletal deformities and short stature. In a small percentage of cases exostoses have exhibited malignant transformation resulting in an osteosarcoma or chondrosarcoma. Osteochondromas development can also occur as a sporadic event. Defects in EXT2 are a cause of Potocki-Shaffer syndrome (POSHS). It is a contiguous gene syndrome due to proximal deletion of chromosome 11p11.2, including EXT2 and ALX4. Belongs to the glycosyltransferase 47 family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Membrane protein, integral; Glycan Metabolism - heparan sulfate biosynthesis; EC 2.4.1.225; EC 2.4.1.224; Tumor suppressor; Transferase
Chromosomal Location of Human Ortholog: 11p12-p11
Cellular Component: Golgi membrane; Golgi apparatus; endoplasmic reticulum membrane; membrane; endoplasmic reticulum; integral to membrane
Molecular Function: acetylglucosaminyltransferase activity; transferase activity, transferring glycosyl groups; protein homodimerization activity; glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity; glucuronosyltransferase activity; protein heterodimerization activity; heparan sulfate N-acetylglucosaminyltransferase activity; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity; metal ion binding
Biological Process: glycosaminoglycan biosynthetic process; ossification; cellular polysaccharide biosynthetic process; heparan sulfate proteoglycan biosynthetic process, polysaccharide chain biosynthetic process; mesoderm formation; glycosaminoglycan metabolic process; carbohydrate metabolic process; heparan sulfate proteoglycan biosynthetic process; protein amino acid glycosylation; pathogenesis; signal transduction; cell differentiation
Disease: Exostoses, Multiple, Type Ii
Research Articles on EXT2
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Product Notes
The EXT2 ext2 (Catalog #AAA9215203) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 182-209. The EXT2 Antibody (Center) reacts with Human (Predicted Reactivity: Bovine, Mouse) and may cross-react with other species as described in the data sheet. AAA Biotech's EXT2 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Immunohistochemistry-Paraffin (IHC-P), Immunofluorescence (IF), Western Blot (WB), Flow Cytometry (FC/FACS). WB~~1:1000 IHC-P~~1:10~50 IF~~1:10~50 FC~~1:10~50. Researchers should empirically determine the suitability of the EXT2 ext2 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 "EXT2, 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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