| 产品详情 |
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| Antigenic Specificity | EXT1 |
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| Clone | polyclonal |
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| Host Species | Rabbit |
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| Reactive Species | human, mouse |
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| Isotype | n/a |
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| Format | affinity purified |
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| Size | 50 µl, 100 µl, 200 µl |
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| Concentration | > 1 mg/ml |
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| Applications | WB, IF/ICC. RUO |
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| Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
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| Description | 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. |
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| Immunogen | Recombinant protein of human EXT1. |
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| Other Names | Exostosin-1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransf; EXT1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Multiple exostoses protein 1; Putative tumor suppressor protein EXT1; EXT; LGS; TTV; LGCR; TRPS2 |
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| Gene, Accession # | EXT1, Gene ID: 2131, SwissProt: Q16394 |
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| Catalog # | abx001647 |
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| Price | |
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| Order / More Info | EXT1 Antibody from ABBEXA |
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| Product Specific References | n/a |
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