Fig. 3.

Analysis of GSL from selected organs. Neutral (a–c) and acidic (d–f) GSL extracted from kidneys, livers, and hearts of WT, αGalA ^−/−, and αGalA ^−/− /Gb3S ^−/− mice were analyzed by TLC. In all three organs, αGalA deficiency resulted in accumulation of a neutral GSL which ran at the height of Gb3 and was absent in the αGalA ^−/− /Gb3S ^−/− double knockout thus confirming its identity as Gb3 (a–c). Accumulation of galabiosylceramide (Gal ₂ Cer) contributed to the storage in kidneys of αGalA ^−/− mice (a), but was—in agreement with being a product of Gb3S—absent in αGalA ^−/− /Gb3S ^−/− kidneys. Gb3S deficiency resulted in a slight accumulation of its substrate lactosylceramide (LacCer) in kidneys (a). Among the acidic GSL (d—f), no accumulation was observed in αGalA ^−/− mice. Some GSL are represented by multiple bands due to their heterogeneous composition of fatty acids resulting in different running properties. Orcinol staining. Std. GSL standard