ESI-QTOF MS detects reduced dysglycosylation following fucose/galactose treatment in FUT8-CDG.
A) In exemplary pretreatment samples taken from Patient A, ESI-QTOF MS both show severe lack of fucosylation as well as hypogalactosylation based on analysis of selected reporter glycans. B) Following supplementation of 1 g/day fucose, a reduction in hypofucosylated glycans was noted while hypogalactosylated glycans remained elevated. Additional supplementation of 10 g galactose/day in conjunction with an increase of fucose supplementation lead to a further shift towards regular glycosylation and specifically a reduction in hypogalactosylation.