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Exostosin 1 (EXT1) Antibody
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Catalogue No: abx001647
Price:
US$203.00
(Size: 20 µl)
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EXT1 Antibody is a Rabbit Polyclonal antibody against EXT1. 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.
Research Articles on Exostosin 1 (EXT1)
| Target | Exostosin 1 (EXT1) |
| Clonality | Polyclonal |
| Reactivity | Human, Mouse, Rat |
| Tested Applications | WB, IF/ICC |
| Host | Rabbit |
| Recommended dilutions | WB: 1/500 - 1/2000, IF/ICC: 1/50 - 1/200. Optimal dilutions/concentrations should be determined by the end user. |
| Conjugation | Unconjugated |
| Immunogen | A synthetic peptide corresponding to a sequence within amino acids 467-746 of human EXT1. |
| Isotype | IgG |
| Form | Liquid |
| Purification | Purified by affinity chromatography. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| UniProt Primary AC | Q16394 (UniProt, ExPASy) |
| UniProt Secondary AC | B2R7V2, Q9BVI9 |
| UniProt Entry Name | EXT1_HUMAN |
| Gene Symbol | EXT1 |
| GeneID | 2131 |
| NCBI Accession | NP_000118.2 |
| KEGG | hsa:2131 |
| String | 9606.ENSP00000367446 |
| Molecular Weight | Calculated MW: 86 kDa Observed MW: 86 kDa |
| Buffer | PBS, pH 7.3, containing 0.05% Proclin-300, 50% glycerol. |
| Concentration | > 0.2 mg/ml |
| Availability | Shipped within 5-10 working days. |
| Note | This product is for research use only. |