Overview

  • Product name
  • Description
    Rabbit polyclonal to Ext1
  • Host species
    Rabbit
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Cow, Baboon, Chinese hamster, Orangutan
  • Immunogen

    Full length protein corresponding to Human Ext1 aa 1-746.
    Sequence:

    MQAKKRYFILLSAGSCLALLFYFGGLQFRASRSHSRREEHSGRNGLHHPS PDHFWPRFPDALRPFVPWDQLENEDSSVHISPRQKRDANSSIYKGKKCRM ESCFDFTLCKKNGFKVYVYPQQKGEKIAESYQNILAAIEGSRFYTSDPSQ ACLFVLSLDTLDRDQLSPQYVHNLRSKVQSLHLWNNGRNHLIFNLYSGTW PDYTEDVGFDIGQAMLAKASISTENFRPNFDVSIPLFSKDHPRTGGERGF LKFNTIPPLRKYMLVFKGKRYLTGIGSDTRNALYHVHNGEDVVLLTTCKH GKDWQKHKDSRCDRDNTEYEKYDYREMLHNATFCLVPRGRRLGSFRFLEA LQAACVPVMLSNGWELPFSEVINWNQAAVIGDERLLLQIPSTIRSIHQDK ILALRQQTQFLWEAYFSSVEKIVLTTLEIIQDRIFKHISRNSLIWNKHPG GLFVLPQYSSYLGDFPYYYANLGLKPPSKFTAVIHAVTPLVSQSQPVLKL LVAAAKSQYCAQIIVLWNCDKPLPAKHRWPATAVPVVVIEGESKVMSSRF LPYDNIITDAVLSLDEDTVLSTTEVDFAFTVWQSFPERIVGYPARSHFWD NSKERWGYTSKWTNDYSMVLTGAAIYHKYYHYLYSHYLPASLKNMVDQ LANCEDILMNFLVSAVTKLPPIKVTQKKQYKETMMGQTSRASRWADPDHF AQRQSCMNTFASWFGYMPLIHSQMRLDPVLFKDQVSILRKKYRDIERL


    Database link: Q16394

  • Positive control
    • Human liver tissue lysate, Ext1 transfected 293T cell line lysate

Properties

  • Form
    Liquid
  • Storage instructions
    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer
    pH: 7.2
    Constituent: 100% PBS
  • Concentration information loading...
  • Purity
    Protein A purified
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab171817 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 86 kDa.

Target

  • Function
    Glycosyltransferase required for the biosynthesis of heparan-sulfate. The EXT1/EXT2 complex possesses substantially higher glycosyltransferase activity than EXT1 or EXT2 alone. Appears to be a tumor suppressor.
  • Tissue specificity
    Ubiquitous.
  • Pathway
    Protein modification; protein glycosylation.
  • Involvement in disease
    Defects in EXT1 are a cause of hereditary multiple exostoses type 1 (EXT1) [MIM:133700]. EXT is a genetically heterogeneous bone disorder caused by genes segregating on human chromosomes 8, 11, and 19 and designated EXT1, EXT2 and EXT3 respectively. EXT is a dominantly inherited skeletal disorder primarily affecting endochondral bone during growth. The disease is characterized by formation of numerous cartilage-capped, benign bone tumors (osteocartilaginous exostoses or osteochondromas) that are often accompanied by skeletal deformities and short stature. In a small percentage of cases exostoses have exhibited malignant transformation resulting in an osteosarcoma or chondrosarcoma. Osteochondromas development can also occur as a sporadic event.
    Defects in EXT1 are a cause of tricho-rhino-phalangeal syndrome type 2 (TRPS2) [MIM:150230]. A syndrome that combines the clinical features of trichorhinophalangeal syndrome type 1 and multiple exostoses type 1. Affected individuals manifest multiple dysmorphic facial features including large, laterally protruding ears, a bulbous nose, an elongated upper lip, as well as sparse scalp hair, winged scapulae, multiple cartilaginous exostoses, redundant skin, and mental retardation. Note=A chromosomal aberration resulting in the loss of functional copies of TRPS1 and EXT1 has been found in TRPS2 patients.
    Defects in EXT1 are a cause of chondrosarcoma (CHDSA) [MIM:215300]. It is a malignant neoplasm derived from cartilage cells. Chondrosarcomas range from slow-growing non-metastasizing lesions to highly aggressive metastasizing sarcomas.
  • Sequence similarities
    Belongs to the glycosyltransferase 47 family.
  • Cellular localization
    Endoplasmic reticulum membrane. Golgi apparatus membrane. The EXT1/EXT2 complex is localized in the Golgi apparatus.
  • Information by UniProt
  • Database links
  • Alternative names
    • 4-alpha-N-acetylglucosaminyltransferase antibody
    • exostoses (multiple) 1 antibody
    • Exostosin 1 antibody
    • Exostosin glycosyltransferase 1 antibody
    • Exostosin-1 antibody
    • EXT antibody
    • EXT1 antibody
    • EXT1_HUMAN antibody
    • Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase antibody
    • glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan antibody
    • Langer-Giedion syndrome chromosome region antibody
    • LGCR antibody
    • LGS antibody
    • Multiple exostoses protein 1 antibody
    • Multiple exostoses protein 1 homolog antibody
    • N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase antibody
    • Putative tumor suppressor protein EXT1 antibody
    • TRPS2 antibody
    • TTV antibody
    see all

Images

  • Anti-FOXL1 antibody (ab171871) at 1 µg/ml + Human liver tissue lysate at 15 µl

    Secondary
    Goat Anti-Rabbit IgG at 1/7500 dilution

    Developed using the ECL technique.

    Predicted band size: 86 kDa

  • All lanes : Anti-Ext1 antibody (ab171817) at 1 µg/ml

    Lane 1 : Ext1 transfecte 293T cell line lysate
    Lane 2 : Non-transfected lysate

    Lysates/proteins at 15 µl per lane.

    Secondary
    All lanes : Goat Anti-Rabbit IgG at 1/7500 dilution

    Developed using the ECL technique.

    Predicted band size: 86 kDa

References

ab171817 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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