製品の概要

  • 製品名Recombinant Human SGSH protein
  • タンパク質長Full length protein

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号P51688
    • 生物種Human
    • 配列MSCPVPACCALLLVLGLCRARPRNALLLLADDGGFESGAYNNSAIATPHL DALARRSLLFRNAFTSVSSCSPSRASLLTGLPQHQNGMYGLHQDVHHFNS FDKVRSLPLLLSQAGVRTGIIGKKHVGPETVYPFDFAYTEENGSVLQVGR NITRIKLLVRKFLQTQDDRPFFLYVAFHDPHRCGHSQPQYGTFCEKFGNG ESGMGRIPDWTPQAYDPLDVLVPYFVPNTPAARADLAAQYTTVGRMDQGV GLVLQELRDAGVLNDTLVIFTSDNGIPFPSGRTNLYWPGTAEPLLVSSPE HPKRWGQVSEAYVSLLDLTPTILDWFSIPYPSYAIFGSKTIHLTGRSLLP ALEAEPLWATVFGSQSHHEVTMSYPMRSVQHRHFRLVHNLNFKMPFPIDQ DFYVSPTFQDLLNRTTAGQPTGWYKDLRHYYYRARWELYDRSRDPHETQN LATDPRFAQLLEMLRDQLAKWQWETHDPWVCAPDGVLEEKLSPQCQPLHN EL
    • 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Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • 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

Recombinant Human SGSH protein 画像

  • 12.5% SDS-PAGE showing ab116779 at approximately 81.29kDa.
    Stained with Coomassie Blue.

Recombinant Human SGSH protein (ab116779) 使用論文

ab116779 has not yet been referenced specifically in any publications.

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