Recombinant Human HEXA protein (ab116804)

製品の概要

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P06865
    • 生物種Human
    • 配列MTSSRLWFSLLLAAAFAGRATALWPWPQNFQTSDQRYVLYPNNFQFQYDV SSAAQPGCSVLDEAFQRYRDLLFGSGSWPRPYLTGKRHTLEKNVLVVSVV TPGCNQLPTLESVENYTLTINDDQCLLLSETVWGALRGLETFSQLVWKSA EGTFFINKTEIEDFPRFPHRGLLLDTSRHYLPLSSILDTLDVMAYNKLNV FHWHLVDDPSFPYESFTFPELMRKGSYNPVTHIYTAQDVKEVIEYARLRG IRVLAEFDTPGHTLSWGPGIPGLLTPCYSGSEPSGTFGPVNPSLNNTYEF MSTFFLEVSSVFPDFYLHLGGDEVDFTCWKSNPEIQDFMRKKGFGEDFKQ LESFYIQTLLDIVSSYGKGYVVWQEVFDNKVKIQPDTIIQVWREDIPVNY MKELELVTKAGFRALLSAPWYLNRISYGPDWKDFYVVEPLAFEGTPEQKA LVIGGEACMWGEYVDNTNLVPRLWPRAGAVAERLWSNKLTSDLTFAYERL SHFRCELLRRGVQAQPLNVGFCEQEFEQT
    • 分子量84 kDa including tags
    • 領域1 to 529

特性

Our Abpromise guarantee covers the use of ab116804 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.
    This protein is 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: 0.79% Tris HCl, 0.3% Glutathione

関連情報

  • 別名
    • Beta hexosaminidase alpha chain precursor
    • Beta hexosaminidase subunit alpha
    • Beta N acetylhexosaminidase
    • Beta N acetylhexosaminidase subunit alpha
    • Beta-hexosaminidase A
    • Beta-hexosaminidase subunit alpha
    • Beta-N-acetylhexosaminidase subunit alpha
    • Hexa
    • HEXA_HUMAN
    • Hexosaminidase A
    • Hexosaminidase A (alpha polypeptide)
    • Hexosaminidase A alpha polypeptide
    • Hexosaminidase subunit A
    • MGC99608
    • N acetyl beta glucosaminidase
    • N acetyl beta glucosaminidase subunit alpha
    • N-acetyl-beta-glucosaminidase subunit alpha
    • TSD
    see all
  • 機能Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity.
  • 関連疾患Defects in HEXA are the cause of GM2-gangliosidosis type 1 (GM2G1) [MIM:272800]; also known as Tay-Sachs disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G1 is characterized by GM2 gangliosides accumulation in the absence of HEXA activity, leading to neurodegeneration and, in the infantile form, death in early childhood. GM2G1 has an increased incidence among Ashkenazi Jews and French Canadians in eastern Quebec. It exists in several forms: infantile (most common and most severe), juvenile and adult (late onset).
  • 配列類似性Belongs to the glycosyl hydrolase 20 family.
  • 翻訳後修飾N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).
  • 細胞内局在Lysosome.
  • Information by UniProt

Recombinant Human HEXA protein 画像

  • 12.5% SDS-PAGE showing ab116804 at approximately 83.93kDa.
    Stained with Coomassie Blue.

Recombinant Human HEXA protein (ab116804) 使用論文

ab116804 has not yet been referenced specifically in any publications.

Product Wall

Thank you for contacting us.

In fact, this product only includes alpha subunit but not beta subunit.

Full-length, here, is not the whole protein, means full-length of ORF ( AAH18927, 1 a.a. - 529 a.a.)

The molecular weight...

Read More

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