Recombinant Human Galactosidase alpha protein (ab114549)

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P06280
    • 生物種Human
    • 配列MQLRNPELHLGCALALRFLALVSWDIPGARALDNGLARTPTMGWLHWERF MCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQR DSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFG YYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVY SCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQ ERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDL RHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWA VAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWT SRLRSHINPTGTVLLQLENTMQMSLKDLL
    • 分子量73 kDa including tags
    • 領域1 to 429

特性

Our Abpromise guarantee covers the use of ab114549 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

    ELISA

    SDS-PAGE

  • 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: 0.3% Glutathione, 0.79% Tris HCl

関連情報

  • 別名
    • AGAL_HUMAN
    • Agalsidase alfa
    • Alpha D galactosidase A
    • Alpha D galactoside galactohydrolase
    • Alpha D galactoside galactohydrolase 1
    • Alpha gal A
    • Alpha galactosidase A
    • Alpha-D-galactosidase A
    • Alpha-D-galactoside galactohydrolase
    • Alpha-galactosidase A
    • GALA
    • Galactosidase, alpha
    • GLA
    • GLA protein
    • Melibiase
    see all
  • 関連疾患Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.
  • 配列類似性Belongs to the glycosyl hydrolase 27 family.
  • 細胞内局在Lysosome.
  • Information by UniProt

Recombinant Human Galactosidase alpha protein 画像

  • 12.5% SDS-PAGE Stained with Coomassie Blue showing ab114549

Recombinant Human Galactosidase alpha protein (ab114549) 使用論文

ab114549 has not yet been referenced specifically in any publications.

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