Fluorogenic Assay for α-Gal A Activity

All materials were purchased as described in the preliminary studies [7 (link),9 (link),13 (link)]. In brief, HEK293H cells, culture medium, all supplements, pcDNA3.1/v5-His TOPOplasmid vector, and the transfection reagent were purchased from Thermo Fisher Scientific (Carlsbad, CA, USA). Additionally, 1-deoxygalactonojirimycin hydrochloride and the synthetic fluorogenic substrate 4-methylumbelliferyl-α-d-galactopyranoside (4-MUG) for α-Gal A activity measurement were purchased from Sigma Aldrich (Steinheim, Germany).

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Lukas J., Cimmaruta C., Liguori L., Pantoom S., Iwanov K., Petters J., Hund C., Bunschkowski M., Hermann A., Cubellis M.V, & Rolfs A. (2020). Assessment of Gene Variant Amenability for Pharmacological Chaperone Therapy with 1-Deoxygalactonojirimycin in Fabry Disease. International Journal of Molecular Sciences, 21(3), 956.

Publication 2020

HEK293H cells

1 deoxygalactonojirimycin 4 mug Cells Culture medium Fluorogenic substrate Galactopyranoside Supplements Transfection Vector

Corresponding Organization : University of Rostock

Other organizations : University of Campania "Luigi Vanvitelli", Centogene (Germany), German Center for Neurodegenerative Diseases, Federico II University Hospital

Top 5 similar protocols

Variable analysis

independent variables

1-deoxygalactonojirimycin hydrochloride

dependent variables

α-Gal A activity measurement

control variables

HEK293H cells
Culture medium
All supplements
PcDNA3.1/v5-His TOPOplasmid vector
Transfection reagent
Synthetic fluorogenic substrate 4-methylumbelliferyl-α-D-galactopyranoside (4-MUG)

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