EXT1 Antibody

• Gene Name:

  Exostosin glycosyltransferase 1

• Gene Aliases:

  EXT, LGS, TTV, LGCR, TRPS2

• Gene ID:

  2131

• Swiss Prot:

  Q16394

• Accession Number:

  NP_000118.2

• Reactivity:

  Human, Mouse, Rat

• Immunogen:

  Recombinant protein of human EXT1.

• Target:

  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.

• Clonality:

  Polyclonal

• Conjugation:

  Unconjugated

• Type:

  Polyclonal Antibody

• Applications:

  WB, IF

• Purification:

  Affinity purified against immunogen.

• Assay Protocol:

  Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Antibody Array (AA) Protocol

• Format:

  Liquid.// PBS with 0.02% sodium azide, 50% glycerol, pH 7.3

• Reconstitution:

  Store at -20C. Avoid repeated freeze/thaw cycles.

• Molecular Weight:

  86 kDa

• Fragment:

  IgG

• NCBI Gene Symbol:

  EXT1

• Host or Source:

  Rabbit

• Protein Name:

  Exostosin-1

• Gene Name URL:

  EXT1

• CAS Number:

  9007-83-4

• Nucleotide Accession Number:

  NM_000127.2