Exostosin 1 (EXT1) Antibody

Catalogue No: abx001647
Price: £120.00
(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.

Target EXT1
Clonality Polyclonal
Reactivity Human, Mouse
Tested Applications WB, IF/ICC
Host Rabbit
Recommended dilutions WB: 1/500 - 1/2000, IF/ICC: 1/10 - 1/100. Optimal dilutions/concentrations should be determined by the end user.
Conjugation Unconjugated
Immunogen Recombinant protein 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
KEGG hsa:2131
String 9606.ENSP00000367446
Molecular Weight Calculated MW: 86 kDa
Observed MW: 88 kDa
Buffer PBS, pH 7.3, 0.02% sodium azide, 50% glycerol.
Concentration > 0.5 mg/ml
Availability Shipped within 5-10 working days.
Note This product is for research use only.
Research Articles on EXT1

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