This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.
product summary
company name :
Invitrogen
other brands :
NeoMarkers, Lab Vision, Endogen, Pierce, BioSource International, Zymed Laboratories, Caltag, Molecular Probes, Research Genetics, Life Technologies, Applied Biosystems, GIBCO BRL, ABgene, Dynal, Affinity BioReagents, Nunc, Invitrogen, NatuTec, Oxoid, Richard-Allan Scientific, Arcturus, Perseptive Biosystems, Proxeon, eBioscience
product type :
antibody
product name :
EXT1 Polyclonal Antibody
catalog :
PA5-76041
quantity :
100 uL
price :
US 464.00
clonality :
polyclonal
host :
domestic rabbit
conjugate :
nonconjugated
reactivity :
human, mouse, rat
application :
immunohistochemistry, immunocytochemistry, immunohistochemistry - paraffin section
product information
Product Type :
Antibody
Product Name :
EXT1 Polyclonal Antibody
Catalog # :
PA5-76041
Quantity :
100 uL
Price :
US 464.00
Clonality :
Polyclonal
Purity :
Antigen affinity chromatography
Host :
Rabbit
Reactivity :
Human, Mouse, Rat
Applications :
ELISA: 1:5,000-1:10,000, Immunocytochemistry: 1:100-1:500, Immunohistochemistry (Paraffin): 1:100-1:500
Species :
Human, Mouse, Rat
Isotype :
IgG
Storage :
Store at 4 C short term. For long term storage, store at -20 C, avoiding freeze/thaw cycles.
Description :
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.
Immunogen :
KLH conjugated synthetic peptide derived from human EXT1: 651-746.
Format :
Liquid
Applications w/Dilutions :
ELISA: 1:5,000-1:10,000, Immunocytochemistry: 1:100-1:500, Immunohistochemistry (Paraffin): 1:100-1:500
Aliases :
AA409028; exostoses (multiple) 1; exostosin 1; exostosin glycosyltransferase 1; exostosin-1; EXT; Ext1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase; glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Langer-Giedion syndrome chromosome region; LGCR; LGS; multiple exostoses protein 1; Multiple exostoses protein 1 homolog; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase; putative tumor suppressor protein EXT1; TRPS2; TTV
company information
Invitrogen
Thermo Fisher Scientific
81 Wyman Street
Waltham, MA USA 02451
https://www.thermofisher.com81 Wyman Street
Waltham, MA USA 02451
800-678-5599
headquarters: USA
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