Recombinant人Galactosidase alpha protein (ab114549)

概述

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      MQLRNPELHLGCALALRFLALVSWDIPGARALDNGLARTPTMGWLHWERF MCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQR DSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFG YYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVY SCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQ ERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDL RHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWA VAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWT SRLRSHINPTGTVLLQLENTMQMSLKDLL
    • 分子量
      73 kDa including tags
    • 氨基酸
      1 to 429

技术指标

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.

  • 应用

    Western blot

    ELISA

    SDS-PAGE

  • 形式
    Liquid
  • 补充说明
    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...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • 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
  • 疾病相关
    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.
  • 序列相似性
    Belongs to the glycosyl hydrolase 27 family.
  • 细胞定位
    Lysosome.
  • Information by UniProt

图片

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

文献

ab114549 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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