EXT1 polyclonal Antibody | BS6597

EXT1 polyclonal Antibody | BS6597 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human,Mouse

Application: WB IF

Application Range: WB 1:500 - 1:2000 IF 1:50 - 1:200

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.

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.

Molecular Weight: ~ 86 kDa

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: 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: Recombinant full length Human EXT1.

Conjugate: Unconjugated

Modification: Unmodification

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) .

Pathway: N/A