EXT2, Polyclonal Antibody

Exostosin-2; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Multiple exostoses protein 2; Putative tumor suppressor protein EXT2; EXT2

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

RB23548

Rabbit

This EXT2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 182-209 amino acids from the Central region of human EXT2.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-EXT2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

EXT2 encodes one of two glycosyltransferases involved in the chain elongation step of heparan sulfate biosynthesis.

Crown Antibodies; Signal Transduction; Stem Cells

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

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.

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.

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).

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.

82255

exostosin glycosyltransferase 2

exostosin-2

Exostosin-2

EXT2_HUMAN

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]

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

Zhao, J., et al. Diabetes 59(3):751-755(2010)
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)

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