Overview

Description

  • Nature

    Recombinant
  • Source

    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species

      Escherichia coli
    • Sequence

      MGSSHHHHHH SSGLVPRGSH MGSMMSAPKI TFIGAGSTIF VKNILGDVFH REALKTAHIA LMDIDPTRLE ESHIVVRKLM DSAGASGKIT CHTQQKEALE DADFVVVAFQ IGGYEPCTVT DFEVCKRHGL EQTIADTLGP GGIMRALRTI PHLWQICEDM TEVCPDATML NYVNPMAMNT WAMYARYPHI KQVGLCHSVQ GTAEELARDL NIDPATLRYR CAGINHMAFY LELERKTADG SYVNLYPELL AAYEAGQAPK PNIHGNTRCQ NIVRYEMFKK LGYFVTESSE HFAEYTPWFI KPGREDLIER YKVPLDEYPK RCVEQLANWH KELEEYKKAS RIDIKPSREY ASTIMNAIWT GEPSVIYGNV RNDGLIDNLP QGCCVEVACL VDANGIQPTK VGTLPSHLAA LMQTNINVQT LLTEAILTEN RDRVYHAAMM DPHTAAVLGI DEIYALVDDL IAAHGDWLPG WLHR
    • Molecular weight

      53 kDa including tags
    • Amino acids

      1 to 451
    • Tags

      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab126658 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

  • Purity

    > 90 % SDS-PAGE.
    ab126658 is purified using conventional chromatography techniques
  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 0.58% 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

Images

  • 15% SDS-PAGE showing ab126658 (3µg).

References

ab126658 has not yet been referenced specifically in any publications.

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