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• Mixture of substrate and internal standard for 50 MS/MS enzyme assay of Acid α-glucosidase (GAA).

 

• Comes with the relevant buffer.

 

• Comes with qNMR quantified enzymatic product relevant to the GAA assay, for mass spectrometry tuning and standard curve generation (See Absolute Quantification).

 

 

 

Pompe

Acid α-Glucosidase Assay Cocktail

SKU: CS5
  • The mixture contains product numbers: SM34B (MW=659.77) and HA28A (MW=502.66).

     

    Catalog Number: CS5

    1. Elliott, S., Buroker, N., Cournoyer, J.J., Potier, A.M., Trometer, J.D., Elbin, C., Schermer, M.J., Kantola, J., Boyce, A., Turecek, F. and Gelb, M.H., 2016. Pilot study of newborn screening for six lysosomal storage diseases using Tandem Mass Spectrometry. Molecular genetics and metabolism, 118(4), pp.304-309.
    2. Elliott, S., Buroker, N., Cournoyer, J.J., Potier, A.M., Trometer, J.D., Elbin, C., Schermer, M.J., Kantola, J., Boyce, A., Turecek, F. and Gelb, M.H., 2016. Dataset and standard operating procedure for newborn screening of six lysosomal storage diseases: By tandem mass spectrometry. Data in brief, 8, pp.915-924.
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