abx001647

antibody from Abbexa

Targeting: EXT1 LGCR, LGS, ttv

Western blot (Recommended by provider)

Immunocytochemistry (Recommended by provider)

Antibody Data

Product number

abx001647

Provider

Abbexa

Product name

Exostosin 1 (EXT1) Antibody

Antibody type

Polyclonal

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.

Reactivity

Human, Mouse

Host

Rabbit

Conjugate

Unconjugated

Isotype

IgG

Vial size

100 µl

Concentration

> 1 mg/ml

Storage

Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.