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Invitrogen
EXT1 Polyclonal Antibody
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FIGURE: 1 / 6
EXT1 Antibody (PA5-106907) in IHC (P)
Immunohistochemistry analysis of EXT1 in mouse muscle tissue. The sample was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. Samples were incubated with EXT1 polyclonal antibody (Product # PA5-106907) using a dilution of 1:100 (4°C overnight) followed by HRP conjugated anti-Rabbit secondary antibody.
Product Details
PA5-106907
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Published species
C. elegans,
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
A synthesized peptide derived from human EXT1(Accession Q16394), corresponding to amino acid residues R6-P56.
Conjugate
Form
Liquid
Concentration
1 mg/mL
Purification
Affinity chromatography
Storage buffer
PBS, pH 7.4, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
Product Specific Information
Antibody detects endogenous levels of total EXT1.
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.
Bioinformatics
Protein Aliases: exostoses (multiple) 1; exostosin 1; Exostosin glycosyltransferase 1; Exostosin-1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase; glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Heparan sulfate co-polymerase subunit EXT1; 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, (Rat) 299907, (Mouse) 14042
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
molecular_function
transferase activity
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
biological_process
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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