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. 2018 Feb 22;19(2):625. doi: 10.3390/ijms19020625

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© 2018 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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Alterations in gangliosides in the cerebellar precursor cells from the Cln3^Δex7/8 mouse model. (a) Quantitative HPLC analysis of glycosphingolipids in wildtype (WT) and Cln3^Δex7/8 cells shows increased levels of GM3, together with a reduction in GM1a and GD1a levels. Statistical analysis with 2-way ANOVA. p values * p < 0.05; *** p < 0.001; (b) Staining of GD1a, GM1a and GM2 in WT and Cln3^Δex7/8 cells. GM1a and GD1a levels are reduced in Cln3^Δex7/8 cells as compared with WT cells. The cells were immunostained with specific antibodies against the respective gangliosides and detected with a secondary antibody coupled to Alexa 488. Blue staining shows the nuclei stained with DAPI. Scale bar: 20 µm.