Invitrogen™ EXT1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA5112922

Catalog No. PIPA5112922

Description

EXT1 Polyclonal Antibody for Western Blot, ELISA

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.

Specifications

• Antigen: EXT1
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: EXT1
• Gene Alias: AA409028; exostoses (multiple) 1; exostosin 1; exostosin glycosyltransferase 1; exostosin-1; EXT; Ext1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase; glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Langer-Giedion syndrome chromosome region; LGCR; LGS; multiple exostoses protein 1; Multiple exostoses protein 1 homolog; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase; putative tumor suppressor protein EXT1; TRPS2; TTV
• Host Species: Rabbit
• Purification Method: Protein G
• Regulatory Status: RUO
• Gene ID (Entrez): 2131
• Content And Storage: -20°C or -80°C if preferred
• Form: Liquid

• Applications: ELISA, Western Blot
• Concentration: 3.25 mg/mL
• Formulation: PBS with 50% glycerol and 0.03% ProClin 300; pH 7.4
• Gene Accession No.: Q16394
• Gene Symbols: EXT1
• Immunogen: Recombinant Human Exostosin-1 protein (28-171AA).
• Quantity: 100 μg
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG

Safety and Handling

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