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Recombinant Human SGSH/HSS protein (ab116779)

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SDS-PAGE - Recombinant Human SGSH/HSS protein (ab116779)

    Key features and details

    • Expression system: Wheat germ
    • Suitable for: WB, SDS-PAGE, ELISA

    製品の詳細

    • 製品名

      Recombinant Human SGSH/HSS protein

    • 発現系

      Wheat germ

    • アクセッション番号

      P51688

    • タンパク質長

      Full length protein

    • Animal free

      No

    • 由来

      Recombinant

      • 生物種

        Human

      • 配列

        MSCPVPACCALLLVLGLCRARPRNALLLLADDGGFESGAYNNSAIATPHL DALARRSLLFRNAFTSVSSCSPSRASLLTGLPQHQNGMYGLHQDVHHFNS FDKVRSLPLLLSQAGVRTGIIGKKHVGPETVYPFDFAYTEENGSVLQVGR NITRIKLLVRKFLQTQDDRPFFLYVAFHDPHRCGHSQPQYGTFCEKFGNG ESGMGRIPDWTPQAYDPLDVLVPYFVPNTPAARADLAAQYTTVGRMDQGV GLVLQELRDAGVLNDTLVIFTSDNGIPFPSGRTNLYWPGTAEPLLVSSPE HPKRWGQVSEAYVSLLDLTPTILDWFSIPYPSYAIFGSKTIHLTGRSLLP ALEAEPLWATVFGSQSHHEVTMSYPMRSVQHRHFRLVHNLNFKMPFPIDQ DFYVSPTFQDLLNRTTAGQPTGWYKDLRHYYYRARWELYDRSRDPHETQN LATDPRFAQLLEMLRDQLAKWQWETHDPWVCAPDGVLEEKLSPQCQPLHN EL

      • 予測される分子量

        81 kDa including tags

      • 領域

        1 to 502

    特性

    Our Abpromise guarantee covers the use of ab116779 in the following tested applications.

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

    • アプリケーション

      Western blot

      SDS-PAGE

      ELISA

    • 製品の状態

      Liquid

    • 備考

      This product was previously labelled as SGSH.

       

    • Concentration information loading...

    前処理および保存

    • 保存方法および安定性

      Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

      pH: 8.00
      Constituents: 0.3% Glutathione, 0.79% Tris HCl

    関連情報

    • 別名

      • Heparan sulfate sulfatase
      • Heparan sulphate sulphatase
      • HSS
      • MPS 3A
      • MPS3 A
      • MPS3A
      • Mucopolysaccharidosis type IIIA
      • N sulfoglucosamine sulfohydrolase
      • N sulfoglucosamine sulfohydrolase (sulfamidase)
      • N-sulphoglucosamine sulphohydrolase
      • SFMD
      • SGSH
      • SPHM_HUMAN
      • Sulfoglucosamine sulfamidase
      • Sulphamidase
      • Sulphoglucosamine sulphamidase
      see all

    • 関連疾患

      Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival.

    • 配列類似性

      Belongs to the sulfatase family.

    • 翻訳後修飾

      The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.

    • 細胞内局在

      Lysosome.
    • Information by UniProt

    画像

    • SDS-PAGE - Recombinant Human SGSH/HSS protein (ab116779)
      SDS-PAGE - Recombinant Human SGSH/HSS protein (ab116779)
      12.5% SDS-PAGE showing ab116779 at approximately 81.29kDa.
      Stained with Coomassie Blue.

    プロトコール

    To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.

    Click here to view the general protocols

    参考文献 (0)

    ab116779 を使用した論文を発表された方は、こちらまでお知らせください。データシートに掲載させていただきます。

    ab116779 は論文での使用が確認できていません。

    レビューと Q&A

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