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Home / Primary Antibodies / IHC Antibodies

EXT1 Antibody

SKU : BHA10557293

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CUSABIO TECHONOLOGY LLC

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Mfr.No.	CSB-PA007899KA01HU
Alternative Names	EXT1; EXT; LGS; TTV; LGCR; TRPS2
Categories	Primary Antibodies
Description	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.
Hgnc	3512
Host	Rabbit
Immunogen	Recombinant protein of human EXT1
Intended Use	For research use only. Not for human, diagnostic or therapeutic use.
Involvement In Disease	Hereditary multiple exostoses 1 (EXT1); Tricho-rhino-phalangeal syndrome 2 (TRPS2); Chondrosarcoma (CHDSA)
Isotype	IgG
Mgi	894663
Raised In	Rabbit
Reactivity	Human, Mouse, Rat
Regulatory	RUO
Relevance	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.
Size	100μl(100μg)
Species	Homo Sapiens (Human)
Subcellular Location	Endoplasmic reticulum membrane, Single-pass type II membrane protein, Golgi apparatus membrane, Single-pass type II membrane protein

Rgd	1587375
Target Details	This gene encodes an endoplasmic reticulum-resident type II transmembrane glycosyltransferase involved in the chain elongation step of heparan sulfate biosynthesis. Mutations in this gene cause the type I form of multiple exostoses.
Uniprot	Q16394
Database Links	HGNC:3512 OMIM:133700 KEGG:hsa:2131 STRING:9606.ENSP00000367446 UniGene:Hs.492618
Function	Glycosyltransferase required for the biosynthesis of heparan-sulfate. The EXT1/EXT2 complex possesses substantially higher glycosyltransferase activity than EXT1 or EXT2 alone. Appears to be a tumor suppressor. Required for the exosomal release of SDCBP, CD63 and syndecan
Protein Families	Glycosyltransferase 47 family
Tissue Specificity	Ubiquitous.

Buffer	Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Format	liquid
Purification	Affinity purification
Purity	Affinity purification
Storage	Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Storage Buffer	Store at -20oC or -80oC. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Applications

ELISA, WB, IHC

Description

ELISA ; WB ; IHC, WB ; ELISA ; IHC

Dilution

WB	1:500-1:2000
IHC	1:50-1:200

Tested Application

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

Datasheet
ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol

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SKU	Supplier No.	Size	Your price	Quantity
BHA10557293	CSB-PA007899KA01HU	100μl	$387.33		Add to Cart

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