Overview

Description

  • Nature

    Recombinant
  • Source

    Mammalian
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      LDNGLARTPTMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEGW KDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKL GIYADVGNKTCAGFPGSFGYYDIDAQTFADWGVDLLKFDGCYCDSLENLA DGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFAD IDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQV TQMALWAIMAAPLFMSNDLRHISPQAKALLQDKDVIAINQDPLGKQGYQL RQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPA CFITQLLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTMQMSLKDLLVD HHHHHH
    • Molecular weight

      46 kDa including tags
    • Amino acids

      32 to 429
    • Tags

      His tag C-Terminus

Specifications

Our Abpromise guarantee covers the use of ab151641 in the following tested applications.

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

  • Applications

    SDS-PAGE

    HPLC

  • Endotoxin level

    < 1.000 Eu/µg
  • Purity

    > 95 % SDS-PAGE.
    ab151641 is greater than 95% pure as determined by SEC-HPLC and reducing SDS-PAGE. Supplied as a 0.2 µm filtered solution.
  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.32% Tris HCl, 0.88% Sodium chloride

General Info

  • Alternative names

    • 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
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the glycosyl hydrolase 27 family.
  • Cellular localization

    Lysosome.
  • Information by UniProt

References

ab151641 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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