EXT1 Antibody | CSB-PA007899KA01HU

EXT1 Antibody | CSB-PA007899KA01HU | Cusabio

EXT1 Antibody is Available at Gentaur Genprice with the fastest delivery.

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Product Type: Polyclonal Antibody

Target Names: EXT1

Aliases: EXT1; EXT; LGS; TTV; LGCR; TRPS2

Background: 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.

Isotype: IgG

Conjugate: Non-conjugated

Clonality: Polyclonal

Uniport ID: Q16394

Host Species: Rabbit

Species Reactivity: Human, Mouse, Rat

Immunogen: Recombinant protein of human EXT1

Immunogen Species: Human

Applications: ELISA, WB, IHC

Tested Applications: ELISA, WB, IHC;WB:1:500-1:2000, IHC:1:50-1:200

Purification Method: Affinity purification

Dilution Ratio1: ELISA:1:2000-1:10000

Dilution Ratio2: WB:1:500-1:2000

Dilution Ratio3: IHC:1:50-1:200

Dilution Ratio4:

Dilution Ratio5:

Dilution Ratio6:

Buffer: Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Form: liquid

Storage: Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Initial Research Areas: Signal Transduction

Research Areas: Signal transduction