EXT1 polyclonal antibody

• 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.

• 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

• CAS Number:

  9007-83-4