EXT2 Antibody | MyBioSource MBS2524268 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

EXT2 Antibody

catalog :

MBS2524268

quantity :

0.06 mL

price :

195 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot, ELISA, immunohistochemistry

more info or order :

MyBioSource product webpage

image

image 1 :

Western Blot analysis of Jurkat cells, using EXT2 Polyclonal Antibody at dilution of 1:1000.

image 2 :

Immunohistochemistry of paraffin-embedded human colon cancer using EXT2 Polyclonal Antibody at dilution of 1:50.

product information

catalog number :

MBS2524268

products type :

Antibody

products full name :

EXT2 Antibody

products short name :

[EXT2]

products name syn :

[exostoses (multiple) 2, Exostosin 2, EXT2, Multiple exostoses protein 2, SOTV]

other names :

[exostosin-2 isoform 3; Exostosin-2; exostosin-2; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase; glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; multiple exostoses protein 2; putative tumor suppressor protein EXT2; exostosin glycosyltransferase 2; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Multiple exostoses protein 2; Putative tumor suppressor protein EXT2]

products gene name :

[EXT2]

other gene names :

[EXT2; EXT2; SOTV]

uniprot entry name :

EXT2_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human, Mouse, Rat

purity :

Affinity Purification

form :

PBS with 0.1% sodium azide, 50% glycerol, pH7.3

storage stability :

Store at -20°C, avoid freeze / thaw cycles.

tested application :

Western Blot (WB), Immunohistochemistry (IHC), ELISA

app notes :

WB 1:500-1:2000, IHC 1:20-1:200

image1 heading :

Western Blot (WB)

image2 heading :

Immunohistochemistry (IHC)

other info1 :

Immunogen: Fusion protein of human EXT2.

products description :

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. Required for the exosomal release of SDCBP, CD63 and syndecan.

ncbi gi num :

296010875

ncbi acc num :

NP_001171554.1

ncbi gb acc num :

NM_001178083.1

ncbi mol weight :

Calculated MW: 82kDa. Observed MW: 70-82kDa

ncbi pathways :

Defective B3GAT3 Causes JDSSDHD Pathway (1127588); Defective B4GALT1 Causes B4GALT1-CDG (CDG-2d) Pathway (1127592); Defective B4GALT7 Causes EDS, Progeroid Type Pathway (1127587); Defective CHST14 Causes EDS, Musculocontractural Type Pathway (1127591); Defective CHST3 Causes SEDCJD Pathway (1127590); Defective CHST6 Causes MCDC1 Pathway (1127593); Defective CHSY1 Causes TPBS Pathway (1127589); Defective EXT1 Causes Exostoses 1, TRPS2 And CHDS Pathway (1127594); Defective EXT2 Causes Exostoses 2 Pathway (1127595); Defective PAPSS2 Causes SEMD-PA Pathway (1127586)

ncbi summary :

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 summary :

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: Glycan Metabolism - heparan sulfate biosynthesis; EC 2.4.1.225; Tumor suppressor; EC 2.4.1.224; Transferase; Membrane protein, integral. 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; protein heterodimerization activity; glucuronosyltransferase activity; heparan sulfate N-acetylglucosaminyltransferase activity; metal ion binding; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity. Biological Process: glycosaminoglycan biosynthetic process; ossification; cellular polysaccharide biosynthetic process; heparan sulfate proteoglycan biosynthetic process, polysaccharide chain biosynthetic process; mesoderm formation; glycosaminoglycan metabolic process; heparan sulfate proteoglycan biosynthetic process; carbohydrate metabolic process; protein amino acid glycosylation; pathogenesis; cell differentiation; signal transduction. Disease: Exostoses, Multiple, Type Ii

size1 :

0.06 mL

price1 :

195 USD

size2 :

0.12 mL

price2 :

270

size3 :

0.2 mL

price3 :

435

more info or order :

MyBioSource product webpage