EXT1 Antibody

CAT: 0247-OAAN00678-100ULSize: 100 µLDry Ice: NoHazardous: No
CAT#:0247-OAAN00678-100ULSize:100 µL
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CAS Number
9007-83-4
Gene Name
Exostosin glycosyltransferase 1
Gene Aliases
EXT, LGS, TTV, LGCR, TRPS2
Gene ID
2131
Swiss Prot
Q16394
Accession Number
NP_000118.2
Reactivity
Human, Mouse, Rat
Immunogen
Recombinant protein of human EXT1.
Target
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.
Clonality
Polyclonal
Conjugation
Unconjugated
Type
Polyclonal Antibody
Applications
WB, IF
Purification
Affinity purified against immunogen.
Assay Protocol
Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Antibody Array (AA) Protocol
Format
Liquid.// PBS with 0.02% sodium azide, 50% glycerol, pH 7.3
Reconstitution
Store at -20C. Avoid repeated freeze/thaw cycles.
Molecular Weight
86 kDa
Fragment
IgG
NCBI Gene Symbol
EXT1
Host or Source
Rabbit
Protein Name
Exostosin-1
Gene Name URL
EXT1
Nucleotide Accession Number
NM_000127.2

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