Assay-kits

SensoLyte® Blue Glucocerebrosidase (GBA) Activity Assay Kit Fluorimetric - 1 kit

$504.00
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  • Cat.Number : AS-72258
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Glucocerebrosidase/GBA (also called acid β-glucosidase, Glucosylceramidase) is a lysosomal enzyme responsible for the breakdown of glucocerebroside releasing glucose and ceramide. Deficiency of this enzyme due to genetic mutations leads to accumulation of glucocerebroside and development of lysosomal storage disease, known as Gaucher disease (GD). Mutations in the glucocerebrosidase (GBA1) gene are also associated with increased risk for Parkinson disease and related disorders. It has been hypothesized that GBA, when not available to clear out proteins like alpha-synuclein, results in the accumulation of the proteins thereby contributing to Parkinson’s disease.
The SensoLyte® Blue Glucocerebrosidase (GBA) Assay Kit detects GBA activity by using a most common fluorogenic substrate, MUGlc, for measurement of GBA activity. In presence of GBA the colorless substrate is hydrolyzed into 4-methylumbelliferone (4MU), and after addition of stop solution a bright blue fluorescence can be monitored at excitation/emission = 365nm/445nm nm.

Specifications

Packaging
Kits components
  • Component A: GBA substrate Ex/Em=365 nm/445 nm upon cleavage: 100 µL Component B: 4MU, reference standard: 20 µL Component C: GBA enzyme, Human Recombinant: 10 µL Component D: Assay Buffer: 25 mL Component E: GBA inhibitor: 15 µL Component F: Stop solution: 10 mL
Chemistry
UniProt number
  • P04062
Properties
Absorbance (nm)
  • 365
Emission (nm)
  • 445
Storage & stability
Storage Conditions
  • Store component C at -80°C. Store all other components at -20°C. Component D and F can be stored at room temperature for convenience. Protect components A and B from light and moisture.
Activity
Application
Biomarker Target
Detection Method
Research Area
Sub-category Research Area
Usage
  • Research use
Source
Host

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References

Development of targeted therapies for Parkinson’s disease and related synucleinopathies

J Lipid Res . 2014 Oct 01 ; 55(10) 1996 | DOI : 10.1194/jlr.R047381

  • E. Sybertz
  • D. Krainc

Viable Mouse Models of Acid β-Glucosidase Deficiency: The Defect in Gaucher Disease

Am.J.Pathol . 2003 Nov 01 ; 163(5) 2093 | DOI : https://doi.org/10.1016/S0002-9440(10)63566-3

  • Y.H. Xu
  • et al

Multicenter Analysis of Glucocerebrosidase Mutations in Parkinson's Disease

N Engl J Med . 2009 Oct 22 ; 361 1651 | DOI : 10.1056/NEJMoa0901281

  • E. Sidransky
  • et al

Reduced glucocerebrosidase is associated with increased α-synuclein in sporadic Parkinson’s disease

Brain . 2014 Jan 28 ; 137(3) 834 | DOI : https://doi.org/10.1093/brain/awt367

  • KE. Murphy
  • et al