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Product Details
PA5-116952
| Applications | Tested Dilution | Publications |
|---|---|---|
Western Blot (WB) |
1:500-1:2,000 | - |
Immunocytochemistry (ICC/IF) |
1:10-1:100 | - |
| Product Specifications | |
|---|---|
Species Reactivity |
Human, Mouse |
Host/Isotype |
Rabbit / IgG |
Class |
Polyclonal |
Type |
Antibody |
Immunogen |
Recombinant fusion protein containing a sequence corresponding to amino acids 600-700 of human EXT1 |
Conjugate |
Unconjugated |
Form |
Liquid |
Concentration |
0.96 mg/mL |
Purification |
Affinity chromatography |
Storage buffer |
PBS, pH 7.3, with 50% glycerol |
Contains |
0.05% ProClin 300 |
Storage conditions |
-20° C, Avoid Freeze/Thaw Cycles |
RRID |
AB_2901582 |
Product Specific Information
Positive Samples: K-562, SW480, PC-3, Mouse liver, Mouse lung
Target Information
Hereditary multiple exostoses (HME) is an autosomal dominant disorder characterized by the formation of exostoses (EXT), which are cartilage-capped bony protuberances mainly located on long bones. Two proteins associated with EXT, EXT1 and EXT2, form homo/heteromeric complexes in vivo, which leads to the accumulation of both proteins in the Golgi apparatus. EXT1 and EXT2 are endoplasmic reticulum-localized type II transmembrane glycoproteins that possess, or are tightly associated with, glycosyltransferase activities involved in the polymerization of the glycosaminoglycan, heparan sulfate (HS). EXT2 is a protein that harbors the D-glucuronyl (GlcA) and N-acetyl-D-glucosaminyl (GlcNAc) transferase activities required for biosynthesis of HS. EXT1 rescues defective HS biosynthesis and elevates low GlcA and GlcNAc transferase levels in mutated cells.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: exostoses (multiple) 1; exostosin 1; Exostosin-1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Langer-Giedion syndrome chromosome region; Multiple exostoses protein 1; Multiple exostoses protein 1 homolog; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase; Putative tumor suppressor protein EXT1
Gene Aliases: AA409028; EXT; EXT1; LGCR; LGS; TRPS2; TTV
UniProt ID: (Human) Q16394, (Mouse) P97464
Entrez Gene ID: (Human) 2131, (Mouse) 14042
Molecular Function:
glycosyltransferase
skeletal system development
ossification
glycosaminoglycan biosynthetic process
protein glycosylation
signal transduction
gastrulation
axon guidance
endoderm development
mesoderm development
heparan sulfate proteoglycan biosynthetic process
heparan sulfate proteoglycan biosynthetic process, polysaccharide chain biosynthetic process
olfactory bulb development
cellular polysaccharide biosynthetic process
embryonic skeletal joint development
brain development
acetylglucosaminyltransferase activity
glucuronosyltransferase activity
transferase activity, transferring glycosyl groups
heparan sulfate N-acetylglucosaminyltransferase activity
protein homodimerization activity
metal ion binding
protein heterodimerization activity
glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity
transferase activity
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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