Overview

  • Product name

    Alpha Galactosidase Activity Assay Kit
  • Detection method

    Fluorescent
  • Sample type

    Saliva, Urine, Serum, Cell Lysate, Tissue Homogenate
  • Product overview

    Alpha Galactosidase Activity Assay Kit (ab239716) provides a simple, rapid way to monitor total alpha galactosidase(α-Gal) activity in a wide variety of biological samples. In this kit, α-Gal cleaves a synthetic specific substrate releasing a fluorophore, which can be easily quantified (Ex/Em= 360/445 nm). The assay is specific, sensitive and can detect as low as 0.1 μU of α-Galactosidase activity. This kit does not detect beta galactosidase activity.

  • Platform

    Microplate reader

Properties

  • Storage instructions

    Store at -20°C. Please refer to protocols.
  • Components 100 tests
    α-Gal Assay Buffer 1 x 25ml
    α-Gal Stop Buffer 1 x 25ml
    ∝-Gal Positive Control 1 vial
    ∝-Gal Substrate 1 x 220µl
    4-Methylumbelliferone Standard 1 x 35µl
  • Research areas

  • 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
  • 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

Images

Protocols

References

ab239716 has not yet been referenced specifically in any publications.

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