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Increased Gb3 expression and lipid accumulation in GLA-KO human iPSC kidney organoids Next, to determine the accumulation of Gb3, a main pathological mechanism in human Fabry disease 6,7 , confocal microscopy was performed, which revealed extensive accumulation of Gb3 in podocytes and tubular cells in GLA-mutant human iPSC kidney organoids (Fig

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An additional set of aromatic amides with unknown in vivo metabolism were selected using a systematic approach to investigate substituent effects of e.g., electron withdrawing (-NO 2 , -F) and electron donating groups (-OH, -CH 3 ) which were varied between compounds around the formanilide ring ( ortho -, meta - and para - to the amide bond) for future quantitative structure-activity relationship (QSAR) modelling