ARP2131-01 [Polyclonal Antibody]
EXT1 Rabbit Polyclonal Antibody
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Species:   Rabbit
Applications:   WB IHC
Immunogen Range:   A recombinant protein of human EXT1
Clonality:   Polyclonal Antibody
Isotype:   IgG
GENE ID:   2131
Swiss Prot:   Q16394
Synonyms:   EXT, LGS, TTV, LGCR, TRPS2
Purification:   Affinity purification
Storage:   Store at -20oC (regular) or -80oC (long term). Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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.
Caculated MW:   86kDa
Observed MW:   Refer to Figures
Applications:   WB 1:500 - 1:2000
IHC 1:50 - 1:200
Reacitivity:   Human, Mouse, Rat
For research use only. Not intended for diagnostic or therapeutic use!
Additional information