EXT1 Rabbit Polyclonal Antibody

CAT#: TA323730

Anti-EXT1 Rabbit Polyclonal Antibody

Product Images

Predicted band size: 86 kDa. Positive control: MDA-MB231 cell lysate. Recommended dilution: 1/500-2000. (Gel: 8%SDS-PAGE Lysate: 40 ug Primary antibody: 1/5000 dilution Secondary antibody: Goat anti Rabbit IgG - H&L (HRP) at 1/10000 dilution Exposure time: 1 minute)

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  Figure from citation: Immunofluorescence of EXT1 protein level by using anti-EXT1 antibody in hair follicle of EXT1StEpi /StEpi mice. View Citation

Specifications

Product Data

• Applications: IF, WB
• Recommended Dilution: WB: 1:500-2000
• Reactivities: Human, Mouse, Rat
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: Fusion protein corresponding to C terminal 280 amino acids of human exostosin glycosyltransferase 1
• Formulation: PBS pH7.3, 0.05% NaN3, 50% glycerol
• Concentration: lot specific
• Purification: Antigen affinity purification
• Conjugation: Unconjugated
• Storage: Store at -20°C as received.
• Stability: Stable for 12 months from date of receipt.
• Predicted Protein Size: 86 kDa
• Gene Name: exostosin glycosyltransferase 1
• Database Link:
  NP_000118
  Entrez Gene 14042 MouseEntrez Gene 299907 RatEntrez Gene 2131 Human
  UniProt ID Q16394
• Background: Hereditary multiple exostoses (EXT) is an autosomal dominant disorder characterized by the formation of cartilage-capped tumors (exostoses) that develop from the growth plate of endochondral bone. This condition can lead to skeletal abnormalities, short stature and malignant transformation of exostoses to chondrosarcomas or osteosarcomas. Linkage analyses have identified three different genes for EXT, EXT1 on 8q24.1, EXT2 on 11p11-13 and EXT3 on 19p, a family of tumor suppressor genes. Most EXT cases have been attributed to missense or frameshift mutations, which lead to loss of function of the EXT genes. EXT1 is an ER-resident type II transmembrane glycoprotein and a heparan sulphate polymerase with both D-glucuronyl and N-acetyl-D-glucosaminoglycan transferase activities. Expression of EXT1 in cells results in the alteration of the synthesis and display of cell surface heparan sulfate glycosaminoglycans. EXT1 mutations have been identified in multiple types of human tumors.
• Synonyms: EXT; LGCR; LGS; TRPS2; TTV

Reference Data

• Protein Families: Druggable Genome, Transmembrane
• Protein Pathways: Heparan sulfate biosynthesis, Metabolic pathways