EXT1 polyclonal antibody

CAT: 0384-BS6597-01Size: 50 µLDry Ice: NoHazardous: No
CAT#:0384-BS6597-01Size:50 µL
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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.
CAS Number
9007-83-4
Structure Composition
0.01M TBS (pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Product Name Alternative
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
Swiss Prot
Q16394
Reactivity
Human, Mouse, Rat
Immunogen
KLH conjugated synthetic peptide derived from human EXT1:651-746/746
Conjugation
Unconjugated
Applications
IHC IF ICC ELISA
Dilution
IHC 1:100 - 1:500 IF 1:100 - 1:500 ICC 1:100 - 1:500 ELISA 1:5000 - 1:10000
Purity
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .
Modification
Unmodification
Molecular Weight
~ 86 kDa
Storage Conditions
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Notes
For research use only, not for use in diagnostic procedure.
Specificity
EXT1 polyclonal antibody detects endogenous levels of EXT1 protein.
Host or Source
Rabbit

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