mouse monoclonal IgG1; EXT1 Antibody (A-7) is an IgG1 κ mouse monoclonal EXT1 antibody (also designated EXT1 antibody) that detects the EXT1 protein of mouse, rat and human origin by WB, IP, IF and ELISA. EXT1 Antibody (A-7) is available as both the non-conjugated anti-EXT1 antibody form, as well as multiple conjugated forms of anti-EXT1 antibody, including agarose, HRP, PE, FITC and multiple Alexa Fluor® conjugates. 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.

Free sample available. Limit 3 free samples for each full size Santa Cruz Biotechnology primary antibody ordered. All samples must be different. Customer responsible for shipping costs.