Ibiglustat succinate (Venglustat succinate) is a glucosylceramide (GlcCer) synthase inhibitor that blocks the formation of glucosylceramide.
Ibiglustat succinate can prevent additional globotriaosylceramide(GL-3) accumulation and could serve to ameliorate the abundant levels of this sphingolipid in fabry disease cardiomyocytes.[2]
Venglustat administration signifcantly reduces the accumulation of glycosphingolipids and pathological α-synuclein aggregates in the central nervous system and ameliorated the associated memory defcit. [3]
[1] Zappa F, et al. Curr Opin Cell Biol. 2018 Feb;50:102-116. [2] Itier JM, et al. J Inherit Metab Dis. 2014 Nov;37(6):1013-22. [3] Viel C, et al. Sci Rep. 2021 Oct 22;11(1):20945.
















