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Generation of lyso-glycosphingolipids

From left to right: The lysosomal storage disorders, the gene defect, accumulating lysosomal GSL substrates, and the deacylated lyso-GSL species.

Abbreviations: ASA, Arylsulfatase A; GALC, β-galactosylceramidase; GalCer, galactosylceramide; GBA, acid β-glucosidase; GLA, α-galactosidase A; GLB1, β-galactosidase; GB3, globotriaosylceramide; GlcCer, glucosylceramide; GM1, ganglioside GM1; GM2, ganglioside GM2; HEX, β-hexosaminidase; lyso-GSL, lyso-glycosphingolipid; SO, sphingoid.

(Source: van Eijk M, Ferraz MJ, Boot RG, Aerts JM. Lyso-glycosphingolipids: presence and consequences. Essays Biochem 2020;64[3]:565-78. Creative Commons Attribution 4.0 International [CC BY 4.0] license, creativecommons.org/licenses/by/4.0.)

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