Product Name :EXT1 polyclonal antibody
Reactivity :Human, Mouse, Rat
Applications :IHC IF ICC ELISA
Application_all :IHC 1:100 - 1:500
IF 1:100 - 1:500
ICC 1:100 - 1:500
ELISA 1:5000 - 1:10000
Background :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.
Product :0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Purification&Purity :The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Specificity :EXT1 polyclonal antibody detects endogenous levels of EXT1 protein.
Note :For research use only, not for use in diagnostic procedure.
Alternative Name :Exostosin 1; Exostosin-1; EXT1; EXT1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Multiple exostoses protein 1; Multiple exostoses protein 1 homolog; Putative tumor suppressor protein EXT1
Immunogen :KLH conjugated synthetic peptide derived from human EXT1:651-746/746
Modification :Unmodification
