Invitrogen™ EXT1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA5106907

Catalog No. PIPA5106907

Description

Antibody detects endogenous levels of total EXT1.

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: Affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 14042, 2131, 299907
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot
• Concentration: 1 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.4
• Gene Accession No.: P97464, Q16394
• Gene Symbols: EXT1
• Immunogen: A synthesized peptide derived from human EXT1(Accession Q16394), corresponding to amino acid residues R6-P56.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG

Safety and Handling

WARNING: Cancer - www.P65Warnings.ca.gov