产品名称 Exostosin 1 (EXT1) Antibody from ANTIBODIES-ONLINE GmbH
产品货号 ABIN1683569
产品价格 现货询价,电话:010-67529703
产品规格 50 µg
产品品牌 ANTIBODIES-ONLINE GmbH
产品概述
产品详情
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Antigenic SpecificityExostosin 1 (EXT1)
Clonepolyclonal
Host SpeciesRabbit
Reactive Speciesmouse, rat, human
Isotypen/a
Formataffinity purified
Size50 µg
Concentrationn/a
ApplicationsWestern Blotting (WB),Immunohistochemistry (IHC)
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DescriptionHereditary 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. Synonyms: EXT, LGS, TTV, LGCR, TRPS2,
ImmunogenRecombinant protein of human EXT1
Other NamesAA409028|EXT|LGCR|LGS|TRPS2|TTV|xext1
Gene, Accession #Gene ID: 2131,14042,299907
Catalog #ABIN1683569
Price
Order / More InfoExostosin 1 (EXT1) Antibody from ANTIBODIES-ONLINE GmbH
Product Specific Referencesn/a
产品资料