Rabbit EXT1 Polyclonal Antibody | anti-EXT1 antibody

EXT1 Antibody

Cat. Num. AAA9607420

• Gene Names: EXT1; EXT; LGS; TTV; LGCR; TRPS2
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry, ELISA
• Purity: The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin.
• Synonyms: EXT1; Polyclonal Antibody; EXT1 Antibody; 4-alpha-N-acetylglucosaminyltransferase; exostoses (multiple) 1; Exostosin 1; Exostosin glycosyltransferase 1; Exostosin-1; EXT; EXT1_HUMAN; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan; Langer-Giedion syndrome chromosome region; LGCR; LGS; Multiple exostoses protein 1; Multiple exostoses protein 1 homolog; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase; Putative tumor suppressor protein EXT1; TRPS2; TTV; anti-EXT1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: EXT1 antibody detects endogenous levels of total EXT1
• Purity/Purification: The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin.
• Form/Format: Liquid; Phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1mg/ml (varies by lot)
• Sequence Length: 746

• Applicable Applications for anti-EXT1 antibody: Western Blot (WB), Immunohistochemistry (IHC), ELISA (EIA)
• Application Notes: WB: 1:500-1:2000; IHC: 1:50-1:200
• Immunogen: A synthesized peptide derived from human EXT1
• Subcellular Location: Endoplasmic Reticulum Membrane. Golgi Apparatus Membrane. The EXT1/EXT2 complex is localized in the golgi apparatus.
• Tissue Specificity: Ubiquitous.
• Predicted Cross Reactivity: Pig, Bovine, Horse, Sheep, Dog
• Similarity: Pig (100%), Bovine (100%), Horse (100%), Sheep (100%), Dog (100%)
• Conjugation: Unconjugated
• Preparation and Storage: Store at -20 degree C. Stable for 12 months from date of receipt.

Western Blot (WB)

(Western blot analysis of HepG2 cell lysate, using EXT1 Antibody. The lane on the left is treated with the antigen-specific peptide.)

Western Blot (WB)

(Western blot analysis of extracts from rat brain, using EXT1 Antibody.)

Immunohistochemistry (IHC)

(MBS9607420 at 1/100 staining Human breast cancer tissue by IHC-P. The sample was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. The sample was then blocked and incubated with the antibody for 1.5 hours at 22 degree C. An HRP conjugated goat anti-rabbit antibody was used as the secondary.)

Related Product Information for anti-EXT1 antibody

Description: Hereditary multiple exostoses (EXT) is an autosomal dominant disorder characterized by the formation of cartilage-capped tumors (exostoses) that develop from the growth plate of endochondral bone. This condition can lead to skeletal abnormalities, short stature and malignant transformation of exostoses to chondrosarcomas or osteosarcomas. Linkage analyses have identified three different genes for EXT, EXT1 on 8q24.1, EXT2 on 11p11-13 and EXT3 on 19p, a family of tumor suppressor genes. Most EXT cases have been attributed to missense or frameshift mutations, which lead to loss of function of the EXT genes. EXT1 is an ER-resident type II transmembrane glycoprotein and a heparan sulphate polymerase with both D-glucuronyl and N-acetyl-D-glucosaminoglycan transferase activities. Expression of EXT1 in cells results in the alteration of the synthesis and display of cell surface heparan sulfate glycosaminoglycans. EXT1 mutations have been identified in multiple types of human tumors.
Function: 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 (PubMed:22660413).
Subunit Structure: Forms a homo/hetero-oligomeric complex with EXT2.
Similarity: Belongs to the glycosyltransferase 47 family.

NCBI and Uniprot Product Information

• NCBI GI #: 46370066
• NCBI GeneID: 2131
• NCBI Accession #: NP_000118.2
• NCBI GenBank Nucleotide #: NM_000127.2
• UniProt Accession #: Q16394; Q9BVI9; B2R7V2
• Molecular Weight: Observed: 86 kDa; Predicted: 87 kDa
• NCBI Official Full Name: exostosin-1
• NCBI Official Synonym Full Names: exostosin glycosyltransferase 1
• NCBI Official Symbol: EXT1
• NCBI Official Synonym Symbols: EXT; LGS; TTV; LGCR; TRPS2
• NCBI Protein Information: exostosin-1
• UniProt Protein Name: Exostosin-1
• Protein Family: Extensin
• UniProt Gene Name: EXT1

NCBI Description

This gene encodes an endoplasmic reticulum-resident type II transmembrane glycosyltransferase involved in the chain elongation step of heparan sulfate biosynthesis. Mutations in this gene cause the type I form of multiple exostoses. [provided by RefSeq, Jul 2008]

Uniprot 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 (PubMed:22660413).